Heterogeneous group of immunodeficiency syndromes characterized by hypogammaglobulinemia of most isotypes, variable B-cell defects, and the presence of recurrent bacterial infections.
An immunologic deficiency state characterized by an extremely low level of generally all classes of gamma-globulin in the blood.
A dysgammaglobulinemia characterized by a deficiency of IMMUNOGLOBULIN A.
A dysgammaglobulinemia characterized by a deficiency of IMMUNOGLOBULIN G.
Syndromes in which there is a deficiency or defect in the mechanisms of immunity, either cellular or humoral.
Immunoglobulin preparations used in intravenous infusion, containing primarily IMMUNOGLOBULIN G. They are used to treat a variety of diseases associated with decreased or abnormal immunoglobulin levels including pediatric AIDS; primary HYPERGAMMAGLOBULINEMIA; SCID; CYTOMEGALOVIRUS infections in transplant recipients, LYMPHOCYTIC LEUKEMIA, CHRONIC; Kawasaki syndrome, infection in neonates, and IDIOPATHIC THROMBOCYTOPENIC PURPURA.
An immunologic deficiency state characterized by selective deficiencies of one or more, but not all, classes of immunoglobulins.
A tumor necrosis factor receptor superfamily member found expressed on peripheral B-LYMPHOCYTES. It has specificity for B-CELL MATURATION ANTIGEN and TUMOR NECROSIS FACTOR LIGAND SUPERFAMILY MEMBER 13.
Persistent abnormal dilatation of the bronchi.
Lymphoid cells concerned with humoral immunity. They are short-lived cells resembling bursa-derived lymphocytes of birds in their production of immunoglobulin upon appropriate stimulation.
Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.
A classification of B-lymphocytes based on structurally or functionally different populations of cells.
Enlargement of the spleen.
Conditions resulting from abnormalities in the arteries branching from the ASCENDING AORTA, the curved portion of the aorta. These syndromes are results of occlusion or abnormal blood flow to the head-neck or arm region leading to neurological defects and weakness in an arm. These syndromes are associated with vascular malformations; ATHEROSCLEROSIS; TRAUMA; and blood clots.
A class of immunoglobulin bearing mu chains (IMMUNOGLOBULIN MU-CHAINS). IgM can fix COMPLEMENT. The name comes from its high molecular weight and originally being called a macroglobulin.
A group of disorders having a benign course but exhibiting clinical and histological features suggestive of malignant lymphoma. Pseudolymphoma is characterized by a benign infiltration of lymphoid cells or histiocytes which microscopically resembles a malignant lymphoma. (From Dorland, 28th ed & Stedman, 26th ed)
Reproducible depletion of CD4+ lymphocytes below 300 per cubic millimeter in the absence of HIV infection or other known causes of immunodeficiency. This is a rare, heterogeneous syndrome and does not appear to be caused by a transmissible agent.
Morphologic alteration of small B LYMPHOCYTES or T LYMPHOCYTES in culture into large blast-like cells able to synthesize DNA and RNA and to divide mitotically. It is induced by INTERLEUKINS; MITOGENS such as PHYTOHEMAGGLUTININS, and by specific ANTIGENS. It may also occur in vivo as in GRAFT REJECTION.
The smaller fragment formed when complement C4 is cleaved by COMPLEMENT C1S. It is an anaphylatoxin that causes symptoms of immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE) but its activity is weaker than that of COMPLEMENT C3A or COMPLEMENT C5A.
Gene rearrangement of the B-lymphocyte which results in a substitution in the type of heavy-chain constant region that is expressed. This allows the effector response to change while the antigen binding specificity (variable region) remains the same. The majority of class switching occurs by a DNA recombination event but it also can take place at the level of RNA processing.
A relatively small nodular inflammatory lesion containing grouped mononuclear phagocytes, caused by infectious and noninfectious agents.
The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.
A member of tumor necrosis factor superfamily found on MACROPHAGES; DENDRITIC CELLS and T-LYMPHOCYTES. It occurs as transmembrane protein that can be cleaved to release a secreted form that specifically binds to TRANSMEMBRANE ACTIVATOR AND CAML INTERACTOR PROTEIN; and B CELL MATURATION ANTIGEN.
Lymphocytes responsible for cell-mediated immunity. Two types have been identified - cytotoxic (T-LYMPHOCYTES, CYTOTOXIC) and helper T-lymphocytes (T-LYMPHOCYTES, HELPER-INDUCER). They are formed when lymphocytes circulate through the THYMUS GLAND and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen.
Process of classifying cells of the immune system based on structural and functional differences. The process is commonly used to analyze and sort T-lymphocytes into subsets based on CD antigens by the technique of flow cytometry.
Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions.
An infection caused by an organism which becomes pathogenic under certain conditions, e.g., during immunosuppression.
The type species of LENTIVIRUS and the etiologic agent of AIDS. It is characterized by its cytopathic effect and affinity for the T4-lymphocyte.
A member of the tumor necrosis factor receptor superfamily found on most T-LYMPHOCYTES. Activation of the receptor by CD70 ANTIGEN results in the increased proliferation of CD4-POSITIVE T-LYMPHOCYTES and CD8-POSITIVE T-LYMPHOCYTES. Signaling by the activated receptor occurs through its association with TNF RECEPTOR-ASSOCIATED FACTORS.
The altered state of immunologic responsiveness resulting from initial contact with antigen, which enables the individual to produce antibodies more rapidly and in greater quantity in response to secondary antigenic stimulus.
Congenital, inherited, or acquired anomalies of the CARDIOVASCULAR SYSTEM, including the HEART and BLOOD VESSELS.
A soluble substance elaborated by antigen- or mitogen-stimulated T-LYMPHOCYTES which induces DNA synthesis in naive lymphocytes.
The number of LYMPHOCYTES per unit volume of BLOOD.
Acquired hemolytic anemia due to the presence of AUTOANTIBODIES which agglutinate or lyse the patient's own RED BLOOD CELLS.
A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.
An acquired defect of cellular immunity associated with infection by the human immunodeficiency virus (HIV), a CD4-positive T-lymphocyte count under 200 cells/microliter or less than 14% of total lymphocytes, and increased susceptibility to opportunistic infections and malignant neoplasms. Clinical manifestations also include emaciation (wasting) and dementia. These elements reflect criteria for AIDS as defined by the CDC in 1993.
Species of the genus LENTIVIRUS, subgenus primate immunodeficiency viruses (IMMUNODEFICIENCY VIRUSES, PRIMATE), that induces acquired immunodeficiency syndrome in monkeys and apes (SAIDS). The genetic organization of SIV is virtually identical to HIV.
Ratio of T-LYMPHOCYTES that express the CD4 ANTIGEN to those that express the CD8 ANTIGEN. This value is commonly assessed in the diagnosis and staging of diseases affecting the IMMUNE SYSTEM including HIV INFECTIONS.
Process whereby the immune system reacts against the body's own tissues. Autoimmunity may produce or be caused by AUTOIMMUNE DISEASES.
Surgical procedure involving either partial or entire removal of the spleen.
A critical subpopulation of T-lymphocytes involved in the induction of most immunological functions. The HIV virus has selective tropism for the T4 cell which expresses the CD4 phenotypic marker, a receptor for HIV. In fact, the key element in the profound immunosuppression seen in HIV infection is the depletion of this subset of T-lymphocytes.
Invasion of the host organism by microorganisms that can cause pathological conditions or diseases.
The production of ANTIBODIES by proliferating and differentiated B-LYMPHOCYTES under stimulation by ANTIGENS.
Differentiation antigens residing on mammalian leukocytes. CD stands for cluster of differentiation, which refers to groups of monoclonal antibodies that show similar reactivity with certain subpopulations of antigens of a particular lineage or differentiation stage. The subpopulations of antigens are also known by the same CD designation.
A classification of T-lymphocytes, especially into helper/inducer, suppressor/effector, and cytotoxic subsets, based on structurally or functionally different populations of cells.
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
Invasion of the host RESPIRATORY SYSTEM by microorganisms, usually leading to pathological processes or diseases.
Disorders characterized by proliferation of lymphoid tissue, general or unspecified.
A classification of lymphocytes based on structurally or functionally different populations of cells.
Includes the spectrum of human immunodeficiency virus infections that range from asymptomatic seropositivity, thru AIDS-related complex (ARC), to acquired immunodeficiency syndrome (AIDS).
The theory that infectious agents, symbiotic microorganisms, and parasites are normal stimulants for the maturation of the immune system toward a balanced immune response. The theory predicts that lack of such stimulation leads to allergies and AUTOIMMUNE DISEASES.
Works containing information articles on subjects in every field of knowledge, usually arranged in alphabetical order, or a similar work limited to a special field or subject. (From The ALA Glossary of Library and Information Science, 1983)
Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.
Communicable diseases, also known as infectious diseases, are medical conditions that result from the infection, transmission, or colonization of pathogenic microorganisms like bacteria, viruses, fungi, and parasites, which can be spread from one host to another through various modes of transmission.
The science dealing with the establishment and maintenance of health in the individual and the group. It includes the conditions and practices conducive to health. (Webster, 3d ed)
Infectious diseases that are novel in their outbreak ranges (geographic and host) or transmission mode.

In vivo modulation of cytokine synthesis by intravenous immunoglobulin. (1/340)

We examined the effects of intravenous immunoglobulin (IVIG) on cytokine regulation in vivo using samples taken before and after replacement-dose (200-400 mg/kg) IVIG in a group of patients with common variable immunodeficiency (CVID) and X-linked agammaglobulinaemia (XLA). The intracellular cytokine content of CD4+ and CD8+ lymphocytes, and their CD28+/- subsets, were measured following in vitro activation with phorbol myristate acetate (PMA) and ionomycin. The cytokines IL-2, interferon-gamma (IFN-gamma) and tumour necrosis factor-alpha (TNF-alpha), and the early activation marker CD69, were assessed by four-colour flow cytometry of whole blood cultures taken before and after IVIG infusion. There was a significant increase in IL-2 expression in CD4+ (and CD4+28-) cells and an increase in TNF-alpha expression in CD8+28- cells following IVIG in CVID, but not in XLA patients. IFN-gamma and CD69 expression were not affected by IVIG infusion. This increase in TNF-alpha and IL-2, combined with unchanged IFN-gamma expression, is evidence against the putative 'anti-inflammatory' role of IVIG, and may explain the failure of resolution of granulomata in CVID patients treated with IVIG alone.  (+info)

HLA class II homozygosity confers susceptibility to common variable immunodeficiency (CVID). (2/340)

Most cases of CVID occur sporadically, but familial cases do also occur and 15% of the patients with the disease have first degree relatives with IgA deficiency (IgAD). Our purpose was to study CVID association with HLA class II alleles and to ascertain whether this disease shares a common genetic background with IgAD in our population. Patients with CVID (n = 42), were typed using gene amplification and sequence-specific oligonucleotide probing for HLA-DRB1, DRB3, DQA1 and DQB1 loci and their typing compared with that of 96 IgAD and 334 healthy controls. We observed a positive association between non-Asp residues at position 57 of the HLA-DQbeta chain and CVID, although much weaker than in IgAD. Further, we found an association between CVID and homozygosity for genes encoding HLA class II molecules, especially HLA-DQ, not seen in IgAD. The data support the hypothesis that a restricted diversity of HLA class II molecules may contribute to susceptibility to CVID.  (+info)

Gastric pathology in patients with common variable immunodeficiency. (3/340)

BACKGROUND/AIMS: Common variable immunodeficiency (CVID) is an immunological disorder characterised by defective antibody production. Patients with CVID have a high risk of gastric cancer. It has been suggested that gastric cancer results from an interaction between environmental factors and a genetic predisposition. The role of Helicobacter pylori as an environmental factor in gastric carcinogenesis is of current interest. Moreover, p53 gene mutations have been reported in gastric cancer. This study focuses on the gastric pathology of patients with CVID and correlation with H pylori infection. METHODS: Thirty four consecutive dyspeptic patients with CVID (mean age 49.6 years, range 14-72; 17 men) were included in the study. An upper gastrointestinal endoscopy was performed and biopsy specimens were taken from the antrum, incisura angularis, and gastric body. Biopsies were used for histological assessment, to identify the presence of H pylori, and to evaluate p53 overexpression. RESULTS: H pylori infection was detected in 14/34 (41%) patients. Chronic active gastritis involving both antrum and body was observed more frequently in H pylori positive (79%) than H pylori negative (20%) patients (p = 0.001). Similarly, a histological feature of multifocal atrophic gastritis was found more frequently in infected (50%) than uninfected patients (10%) (p = 0.012). In addition, one case of gastric adenocarcinoma and another of notable dysplasia were observed in the H pylori positive group. Overexpression of p53 was found in six (18%) patients, including one with normal gastric mucosa. CONCLUSIONS: It can be hypothesised that both H pylori and p53 alterations play a role in the gastric carcinogenesis of patients with CVID.  (+info)

Effect of IVIG on the hair regrowth in a common variable immune deficiency patient with alopecia universalis. (4/340)

Common variable immune deficiency (CVID) is associated with a variety of autoimmune diseases. Alopecia universalis (AU), believed to have an autoimmune basis, has been found in 1.6% of patients with CVID. Intravenous immunoglobulin (IVIG) therapy is used in various immunodeficiency disorders including CVID, and benefit has been shown in the therapy of autoimmune diseases. We report a patient with CVID and AU treated with IVIG who experienced significant hair regrowth. An 8-year-old girl with CVID and AU was treated with IVIG 400 mg/kg every 4 weeks. Since her second dose of IVIG, regrowth of eyelashes, eyebrows, body and scalp hair was observed in this patient. At present, about 1 year treat-meant of IVIG, significant hair regrowth is noted with 5-6 cm of scalp hair. We believe that IVIG may be beneficial in the treatment of AU, at least in patients with CVID.  (+info)

Identification of a subset of common variable immunodeficiency patients with impaired B-cell protein tyrosine phosphorylation. (5/340)

The mechanisms responsible for common variable immunodeficiency syndrome (CVID) are as yet unknown. In the present study, we show that the B-cell dysfunction in a subset of CVID patients is caused by defective protein tyrosine phosphorylation (PTP). We demonstrated that the PTP level and immunoglobulin (Ig) secretion malfunctions can be successfully repaired when normal plasma membrane components are implanted into these patients' B cells. Stimulation of CVID patients' peripheral blood mononucleated cells with anti-Ig antibody revealed that 7 of 11 patients had lower PTP levels than those found in the normal donor cells. Plasma membrane implantation to the cells of these patients resulted in elevated PTP levels which reached normal levels upon stimulation with anti-human Ig antibody. The results revealed two distinct groups of CVID patients. The first group included patients whose B cells expressed low PTP levels after Ig stimulation. In these patients the plasma membrane implantation restored the normal PTP level as well as the ability to secrete IgM and/or IgG after B-cell stimulation. In the second group, patients whose B cells expressed a normal PTP level after Ig stimulation, with no restoration of their ability to secrete Ig upon plasma membrane implantation and lipopolysaccharide stimulation. We conclude that the first group has an early signal transduction defect located in the B-cell plasma membrane, while in the second group the defect is located elsewhere.  (+info)

Up-regulation of IL-12 in monocytes: a fundamental defect in common variable immunodeficiency. (6/340)

We show that LPS-stimulated circulating CD14-positive monocytes from patients with common variable immunodeficiency (CVID) express a higher proportion of intracellular IL-12-positive cells than monocytes from patients with X-linked agammaglobulinemia or normal subjects. We used four-color flow cytometry and measured IL-12 with an Ab to the p40 subunit following stimulation with LPS. The raised IL-12 is associated with an increased frequency of IFN-gamma-positive T cells, but not of IFN-gamma-positive CD56+ NK cells. These increases in frequency of cytokine-positive cells are due to a decrease in the absolute numbers of circulating monocytes and T cells that are negative for IL-12 and IFN-gamma, respectively. The increased frequency of IL-12-positive monocytes appears to be selective because TNF-alpha was not increased, and is thus unlikely to reflect a general activation. Chronic infection is also unlikely to explain our data since cells from X-linked agammaglobulinemia patients with a similar Ig deficiency do not show these changes. Our data suggest a fundamental abnormality in the IL-12/IFN-gamma circuit in CVID, with up-regulation of IL-12 being the "primary" factor. This imbalance is likely to skew the immune response away from Ab production and also explains the failure of CVID T cells to make Ag-specific memory cells and the chronic inflammatory and granulomatous complications that are a feature of CVID. This disease appears to be a rare example of a polarized Th1-type response and may in part be due to a genetic defect in the control of IL-12 production.  (+info)

Lymphoid interstitial pneumonitis associated with common variable hypogammaglobulinaemia treated with cyclosporin A. (7/340)

Lymphoid interstitial pneumonitis (LIP) is a rare clinicopathological entity that may be associated with common variable immune deficiency (CVID) and may lead to respiratory failure and death. Some patients may respond to prolonged corticosteroid treatment. We hypothesised that, in view of the predominant T cell nature of LIP, cyclosporin A would be a more appropriate choice of immunosuppressive agent and report the first case of its successful use in a woman with LIP associated with CVID.  (+info)

Long-term outcome of chronic hepatitis C virus infection in primary hypogammaglobulinaemia. (8/340)

The clinical course of HCV infection in patients with primary hypogammaglobulinaemia appears to be more severe than in immunocompetent patients. We studied the long-term course of chronic HCV infection in 20 Norwegian hypogammaglobulinaemia patients with a 13-15 year known history of HCV infection. Twelve of 20 patients developed cirrhosis during the observation period (1984-1999), and the remaining eight also had chronic liver disease verified by liver biopsy in the majority of the cases. Eleven of the 20 patients are dead. Two died following liver transplantation for HCV cirrhosis. Five died due to terminal liver failure without receiving a liver allograft. Two patients died from other causes, but with advanced liver disease contributing to the outcome, while two deaths were unrelated to the HCV infection. Among patients with common variable immunodeficiency (CVI), five out of six are dead. Two patients cleared the hepatitis C virus 3 years following interferon monotherapy, while three patients achieved a sustained response to combination therapy with interferon and ribavirin. Viral load did not seem to have a major impact on disease progression. Our results emphasize the severity of hepatitis C virus infection in patients with hypogammaglobulinaemia. Patients with CVI appear to have the poorest prognosis.  (+info)

Common Variable Immunodeficiency (CVID) is a type of primary immunodeficiency disorder characterized by reduced levels of immunoglobulins (also known as antibodies) in the blood, which makes an individual more susceptible to infections. The term "common" refers to its prevalence compared to other types of immunodeficiencies, and "variable" indicates the variability in the severity and types of symptoms among affected individuals.

Immunoglobulins are proteins produced by the immune system to help fight off infections caused by bacteria, viruses, and other pathogens. In CVID, there is a deficiency in the production or function of these immunoglobulins, particularly IgG, IgA, and/or IgM. This results in recurrent infections, chronic inflammation, and an increased risk of developing autoimmune disorders and cancer.

Symptoms of CVID can include:

1. Recurrent sinus, ear, and lung infections
2. Gastrointestinal issues, such as diarrhea, bloating, and malabsorption
3. Autoimmune disorders, like rheumatoid arthritis, lupus, or inflammatory bowel disease
4. Increased risk of certain cancers, particularly lymphomas
5. Fatigue and poor growth in children
6. Delayed puberty in adolescents
7. Lung damage due to recurrent infections
8. Poor response to vaccinations

The exact cause of CVID is not fully understood, but it is believed to be related to genetic factors. In some cases, a family history of immunodeficiency disorders may be present. Diagnosis typically involves blood tests to measure immunoglobulin levels and other immune system components, as well as genetic testing to identify any known genetic mutations associated with CVID. Treatment usually consists of regular infusions of immunoglobulins to replace the missing antibodies and help prevent infections.

Agammaglobulinemia is a medical condition characterized by a severe deficiency or complete absence of gamma globulins (a type of antibodies) in the blood. This deficiency results from a lack of functional B cells, which are a type of white blood cell that produces antibodies to help fight off infections.

There are two main types of agammaglobulinemia: X-linked agammaglobulinemia (XLA) and autosomal recessive agammaglobulinemia (ARA). XLA is caused by mutations in the BTK gene and primarily affects males, while ARA is caused by mutations in other genes and can affect both males and females.

People with agammaglobulinemia are at increased risk for recurrent bacterial infections, particularly respiratory tract infections such as pneumonia and sinusitis. They may also be more susceptible to certain viral and parasitic infections. Treatment typically involves replacement therapy with intravenous immunoglobulin (IVIG) to provide the patient with functional antibodies.

IgA deficiency is a condition characterized by significantly reduced levels or absence of secretory immunoglobulin A (IgA), an important antibody that plays a crucial role in the immune function of mucous membranes lining the respiratory and gastrointestinal tracts. IgA helps to prevent the attachment and multiplication of pathogens, such as bacteria and viruses, on these surfaces.

In individuals with IgA deficiency, the lack of adequate IgA levels makes them more susceptible to recurrent infections, allergies, and autoimmune disorders. The condition can be asymptomatic or may present with various symptoms, such as respiratory tract infections, gastrointestinal issues, and chronic sinusitis. IgA deficiency is typically diagnosed through blood tests that measure the immunoglobulin levels. While there is no cure for IgA deficiency, treatment focuses on managing symptoms and preventing infections through medications, immunizations, and lifestyle modifications.

IgG deficiency is a type of immunodeficiency disorder characterized by reduced levels of immunoglobulin G (IgG) antibodies in the blood. IgG is the most common type of antibody in our body and plays a crucial role in fighting against infections.

There are four subclasses of IgG (IgG1, IgG2, IgG3, and IgG4), and a deficiency in one or more of these subclasses can lead to recurrent infections, particularly of the respiratory tract, such as sinusitis, bronchitis, and pneumonia. People with IgG deficiency may also be more susceptible to autoimmune diseases and allergies.

IgG deficiency can be inherited or acquired, and it is usually diagnosed through blood tests that measure the levels of IgG and other immunoglobulins in the blood. Treatment typically involves preventing infections through vaccinations, antibiotics to treat infections, and in some cases, replacement therapy with intravenous immunoglobulin (IVIG) to boost the immune system.

Immunologic deficiency syndromes refer to a group of disorders characterized by defective functioning of the immune system, leading to increased susceptibility to infections and malignancies. These deficiencies can be primary (genetic or congenital) or secondary (acquired due to environmental factors, medications, or diseases).

Primary immunodeficiency syndromes (PIDS) are caused by inherited genetic mutations that affect the development and function of immune cells, such as T cells, B cells, and phagocytes. Examples include severe combined immunodeficiency (SCID), common variable immunodeficiency (CVID), Wiskott-Aldrich syndrome, and X-linked agammaglobulinemia.

Secondary immunodeficiency syndromes can result from various factors, including:

1. HIV/AIDS: Human Immunodeficiency Virus infection leads to the depletion of CD4+ T cells, causing profound immune dysfunction and increased vulnerability to opportunistic infections and malignancies.
2. Medications: Certain medications, such as chemotherapy, immunosuppressive drugs, and long-term corticosteroid use, can impair immune function and increase infection risk.
3. Malnutrition: Deficiencies in essential nutrients like protein, vitamins, and minerals can weaken the immune system and make individuals more susceptible to infections.
4. Aging: The immune system naturally declines with age, leading to an increased incidence of infections and poorer vaccine responses in older adults.
5. Other medical conditions: Chronic diseases such as diabetes, cancer, and chronic kidney or liver disease can also compromise the immune system and contribute to immunodeficiency syndromes.

Immunologic deficiency syndromes require appropriate diagnosis and management strategies, which may include antimicrobial therapy, immunoglobulin replacement, hematopoietic stem cell transplantation, or targeted treatments for the underlying cause.

Intravenous Immunoglobulins (IVIG) are a preparation of antibodies, specifically immunoglobulins, that are derived from the plasma of healthy donors. They are administered intravenously to provide passive immunity and help boost the immune system's response in individuals with weakened or compromised immune systems. IVIG can be used for various medical conditions such as primary immunodeficiency disorders, secondary immunodeficiencies, autoimmune diseases, and some infectious diseases. The administration of IVIG can help prevent infections, reduce the severity and frequency of infections, and manage the symptoms of certain autoimmune disorders. It is important to note that while IVIG provides temporary immunity, it does not replace a person's own immune system.

Dysgammaglobulinemia is a medical term that refers to an abnormal gamma globulin or immunoglobulin (antibody) level in the blood. Gamma globulins are proteins that play a crucial role in the immune system and help fight off infections. Immunoglobulins are classified into five types (IgA, IgD, IgE, IgG, and IgM), each with a specific function in the immune response.

In dysgammaglobulinemia, there is an imbalance in the levels of these immunoglobulins, which can be either elevated or decreased. This condition can result from various underlying causes, including genetic disorders, autoimmune diseases, infections, and malignancies that affect the bone marrow or lymphatic system.

Depending on the specific pattern of immunoglobulin levels, dysgammaglobulinemia can be further classified into different types, such as:

1. Hypogammaglobulinemia - a decrease in one or more classes of immunoglobulins
2. Agammaglobulinemia - a severe deficiency or absence of all classes of immunoglobulins
3. Hypergammaglobulinemia - an elevation of one or more classes of immunoglobulins

Dysgammaglobulinemia can lead to increased susceptibility to infections, autoimmune disorders, and other health complications. Therefore, it is essential to identify the underlying cause and provide appropriate treatment to manage the condition and prevent further complications.

The Transmembrane Activator and CAML Interactor protein (also known as TACI or TNFRSF13B) is a type I transmembrane protein that belongs to the tumor necrosis factor receptor superfamily. It is involved in the regulation of immune responses, specifically in the activation and differentiation of B cells, which are a type of white blood cell that plays a central role in the humoral immune response.

TACI has two main ligands, or binding partners: A Proliferation-Inducing Ligand (APRIL) and B cell Activating Factor (BAFF). These ligands bind to TACI and activate downstream signaling pathways that lead to the activation and differentiation of B cells.

Mutations in the TACI gene have been associated with various immune disorders, including common variable immunodeficiency (CVID), a primary immunodeficiency disorder characterized by low levels of antibodies and recurrent infections. Additionally, variations in the TACI gene have been linked to an increased risk of developing autoimmune diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA).

Bronchiectasis is a medical condition characterized by permanent, abnormal widening and thickening of the walls of the bronchi (the airways leading to the lungs). This can lead to recurrent respiratory infections, coughing, and the production of large amounts of sputum. The damage to the airways is usually irreversible and can be caused by various factors such as bacterial or viral infections, genetic disorders, immune deficiencies, or exposure to environmental pollutants. In some cases, the cause may remain unknown. Treatment typically includes chest physiotherapy, bronchodilators, antibiotics, and sometimes surgery.

B-lymphocytes, also known as B-cells, are a type of white blood cell that plays a key role in the immune system's response to infection. They are responsible for producing antibodies, which are proteins that help to neutralize or destroy pathogens such as bacteria and viruses.

When a B-lymphocyte encounters a pathogen, it becomes activated and begins to divide and differentiate into plasma cells, which produce and secrete large amounts of antibodies specific to the antigens on the surface of the pathogen. These antibodies bind to the pathogen, marking it for destruction by other immune cells such as neutrophils and macrophages.

B-lymphocytes also have a role in presenting antigens to T-lymphocytes, another type of white blood cell involved in the immune response. This helps to stimulate the activation and proliferation of T-lymphocytes, which can then go on to destroy infected cells or help to coordinate the overall immune response.

Overall, B-lymphocytes are an essential part of the adaptive immune system, providing long-lasting immunity to previously encountered pathogens and helping to protect against future infections.

Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by the immune system's B cells in response to the presence of foreign substances, such as bacteria, viruses, and toxins. These Y-shaped proteins play a crucial role in identifying and neutralizing pathogens and other antigens, thereby protecting the body against infection and disease.

Immunoglobulins are composed of four polypeptide chains: two identical heavy chains and two identical light chains, held together by disulfide bonds. The variable regions of these chains form the antigen-binding sites, which recognize and bind to specific epitopes on antigens. Based on their heavy chain type, immunoglobulins are classified into five main isotypes or classes: IgA, IgD, IgE, IgG, and IgM. Each class has distinct functions in the immune response, such as providing protection in different body fluids and tissues, mediating hypersensitivity reactions, and aiding in the development of immunological memory.

In medical settings, immunoglobulins can be administered therapeutically to provide passive immunity against certain diseases or to treat immune deficiencies, autoimmune disorders, and other conditions that may benefit from immunomodulation.

B-lymphocytes, also known as B-cells, are a type of white blood cell that plays a central role in the humoral immune response. They are responsible for producing antibodies, which are proteins that help to neutralize or destroy pathogens such as viruses and bacteria.

B-lymphocyte subsets refer to distinct populations of B-cells that can be identified based on their surface receptors and functional characteristics. Some common B-lymphocyte subsets include:

1. Naive B-cells: These are mature B-cells that have not yet been exposed to an antigen. They express surface receptors called immunoglobulin M (IgM) and immunoglobulin D (IgD).
2. Memory B-cells: These are B-cells that have previously encountered an antigen and mounted an immune response. They express high levels of surface immunoglobulins and can quickly differentiate into antibody-secreting plasma cells upon re-exposure to the same antigen.
3. Plasma cells: These are fully differentiated B-cells that secrete large amounts of antibodies in response to an antigen. They lack surface immunoglobulins and do not undergo further division.
4. Regulatory B-cells: These are a subset of B-cells that modulate the immune response by producing anti-inflammatory cytokines and suppressing the activation of other immune cells.
5. B-1 cells: These are a population of B-cells that are primarily found in the peripheral blood and mucosal tissues. They produce natural antibodies that provide early protection against pathogens and help to maintain tissue homeostasis.

Understanding the different B-lymphocyte subsets and their functions is important for diagnosing and treating immune-related disorders, including autoimmune diseases, infections, and cancer.

Splenomegaly is a medical term that refers to an enlargement or expansion of the spleen beyond its normal size. The spleen is a vital organ located in the upper left quadrant of the abdomen, behind the stomach and below the diaphragm. It plays a crucial role in filtering the blood, fighting infections, and storing red and white blood cells and platelets.

Splenomegaly can occur due to various underlying medical conditions, including infections, liver diseases, blood disorders, cancer, and inflammatory diseases. The enlarged spleen may put pressure on surrounding organs, causing discomfort or pain in the abdomen, and it may also lead to a decrease in red and white blood cells and platelets, increasing the risk of anemia, infections, and bleeding.

The diagnosis of splenomegaly typically involves a physical examination, medical history, and imaging tests such as ultrasound, CT scan, or MRI. Treatment depends on the underlying cause and may include medications, surgery, or other interventions to manage the underlying condition.

Aortic arch syndromes are a group of conditions that affect the aortic arch, which is the curved portion of the aorta that arises from the left ventricle of the heart and gives rise to the major branches of the arterial system. These syndromes are typically caused by congenital abnormalities or degenerative changes in the aorta and can result in various complications, such as obstruction of blood flow, aneurysm formation, and dissection.

There are several types of aortic arch syndromes, including:

1. Coarctation of the Aorta: This is a narrowing of the aorta at the point where it leaves the heart, just distal to the origin of the left subclavian artery. It can cause hypertension in the upper extremities and reduced blood flow to the lower extremities.
2. Aortic Arch Aneurysm: This is a localized dilation or bulging of the aorta in the region of the aortic arch. It can lead to dissection, rupture, or embolism.
3. Aortic Arch Dissection: This is a separation of the layers of the aortic wall, which can result from hypertension, trauma, or genetic disorders such as Marfan syndrome. It can cause severe chest pain, shortness of breath, and shock.
4. Kommerell's Diverticulum: This is an outpouching or bulge in the aorta at the origin of the ligamentum arteriosum, which is a remnant of the ductus arteriosus. It can cause compression of the airways or esophagus and increase the risk of dissection or rupture.
5. Abernethy Malformation: This is a rare congenital anomaly in which there is an abnormal connection between the portal vein and systemic venous circulation, leading to the bypass of the liver. It can cause various complications such as hepatic encephalopathy, pulmonary hypertension, and liver tumors.

The diagnosis and management of aortic arch syndromes require a multidisciplinary approach involving cardiologists, radiologists, surgeons, and other specialists. Treatment options may include medications, endovascular procedures, or surgical interventions depending on the severity and location of the lesion.

Immunoglobulin M (IgM) is a type of antibody that is primarily found in the blood and lymph fluid. It is the first antibody to be produced in response to an initial exposure to an antigen, making it an important part of the body's primary immune response. IgM antibodies are large molecules that are composed of five basic units, giving them a pentameric structure. They are primarily found on the surface of B cells as membrane-bound immunoglobulins (mlgM), where they function as receptors for antigens. Once an mlgM receptor binds to an antigen, it triggers the activation and differentiation of the B cell into a plasma cell that produces and secretes large amounts of soluble IgM antibodies.

IgM antibodies are particularly effective at agglutination (clumping) and complement activation, which makes them important in the early stages of an immune response to help clear pathogens from the bloodstream. However, they are not as stable or long-lived as other types of antibodies, such as IgG, and their levels tend to decline after the initial immune response has occurred.

In summary, Immunoglobulin M (IgM) is a type of antibody that plays a crucial role in the primary immune response to antigens by agglutination and complement activation. It is primarily found in the blood and lymph fluid, and it is produced by B cells after they are activated by an antigen.

Pseudolymphoma is a term used to describe a benign reactive lymphoid hyperplasia that mimics the clinical and histopathological features of malignant lymphomas. It is also known as pseudolymphomatous cutis or reactive lymphoid hyperplasia.

Pseudolymphoma can occur in various organs, but it is most commonly found in the skin. It is usually caused by a localized immune response to an antigenic stimulus such as insect bites, tattoos, radiation therapy, or certain medications. The condition presents as a solitary or multiple nodular lesions that may resemble lymphoma both clinically and histologically.

Histologically, pseudolymphoma is characterized by a dense infiltrate of lymphocytes, plasma cells, and other immune cells, which can mimic the appearance of malignant lymphoma. However, unlike malignant lymphomas, pseudolymphomas lack cytological atypia, mitotic activity, and clonal proliferation of lymphoid cells.

Pseudolymphoma is usually a self-limiting condition that resolves spontaneously or with the removal of the antigenic stimulus. However, in some cases, it may persist or recur, requiring further evaluation and treatment to exclude malignant lymphoma.

T-lymphocytopenia, idiopathic CD4-positive, also known as Idiopathic CD4 Lymphopenia (ICL), is a rare medical condition characterized by a significant decrease in the number of CD4+ T lymphocytes in the peripheral blood without an identifiable cause. CD4+ T cells are crucial for immune function and protection against certain types of infections, particularly those caused by viruses such as HIV.

ICL is typically defined as a CD4+ T-lymphocyte count below 300 cells/μL in the absence of HIV infection or any other known immunodeficiency disorder. The exact cause of ICL remains unknown, although it has been associated with genetic factors and autoimmune disorders.

People with ICL may be at increased risk for certain types of infections, such as opportunistic infections, which can occur when the immune system is weakened. However, the severity and frequency of infections in individuals with ICL are generally less than those seen in people with HIV-associated CD4 lymphopenia.

Regular monitoring of CD4+ T-lymphocyte counts and appropriate management of any infections that occur are important for people with ICL to maintain their overall health and well-being.

Lymphocyte activation is the process by which B-cells and T-cells (types of lymphocytes) become activated to perform effector functions in an immune response. This process involves the recognition of specific antigens presented on the surface of antigen-presenting cells, such as dendritic cells or macrophages.

The activation of B-cells leads to their differentiation into plasma cells that produce antibodies, while the activation of T-cells results in the production of cytotoxic T-cells (CD8+ T-cells) that can directly kill infected cells or helper T-cells (CD4+ T-cells) that assist other immune cells.

Lymphocyte activation involves a series of intracellular signaling events, including the binding of co-stimulatory molecules and the release of cytokines, which ultimately result in the expression of genes involved in cell proliferation, differentiation, and effector functions. The activation process is tightly regulated to prevent excessive or inappropriate immune responses that can lead to autoimmunity or chronic inflammation.

Complement C4a is a protein fragment or cleavage product generated during the activation of the complement system, which is a part of the immune system. The complement system helps to eliminate pathogens and damaged cells by marking them for destruction and direct lysis. Complement component 4 (C4) is one of the key proteins in this cascade, and it gets cleaved into C4a and C4b during the activation process.

C4a is a small anaphylatoxin with a molecular weight of approximately 9 kDa. It has chemotactic properties, meaning it can attract immune cells like neutrophils to the site of complement activation. Additionally, C4a can induce histamine release from mast cells and basophils, contributing to local inflammation. However, its precise physiological role in the immune response is not entirely clear, and dysregulation of C4a production has been implicated in several pathological conditions, such as autoimmune diseases and allergies.

Immunoglobulin class switching, also known as isotype switching or class switch recombination (CSR), is a biological process that occurs in B lymphocytes as part of the adaptive immune response. This mechanism allows a mature B cell to change the type of antibody it produces from one class to another (e.g., from IgM to IgG, IgA, or IgE) while keeping the same antigen-binding specificity.

During immunoglobulin class switching, the constant region genes of the heavy chain undergo a DNA recombination event, which results in the deletion of the original constant region exons and the addition of new constant region exons downstream. This switch allows the B cell to express different effector functions through the production of antibodies with distinct constant regions, tailoring the immune response to eliminate pathogens more effectively. The process is regulated by various cytokines and signals from T cells and is critical for mounting an effective humoral immune response.

A granuloma is a small, nodular inflammatory lesion that occurs in various tissues in response to chronic infection, foreign body reaction, or autoimmune conditions. Histologically, it is characterized by the presence of epithelioid macrophages, which are specialized immune cells with enlarged nuclei and abundant cytoplasm, often arranged in a palisading pattern around a central area containing necrotic debris, microorganisms, or foreign material.

Granulomas can be found in various medical conditions such as tuberculosis, sarcoidosis, fungal infections, and certain autoimmune disorders like Crohn's disease. The formation of granulomas is a complex process involving both innate and adaptive immune responses, which aim to contain and eliminate the offending agent while minimizing tissue damage.

Immunoglobulin G (IgG) is a type of antibody, which is a protective protein produced by the immune system in response to foreign substances like bacteria or viruses. IgG is the most abundant type of antibody in human blood, making up about 75-80% of all antibodies. It is found in all body fluids and plays a crucial role in fighting infections caused by bacteria, viruses, and toxins.

IgG has several important functions:

1. Neutralization: IgG can bind to the surface of bacteria or viruses, preventing them from attaching to and infecting human cells.
2. Opsonization: IgG coats the surface of pathogens, making them more recognizable and easier for immune cells like neutrophils and macrophages to phagocytose (engulf and destroy) them.
3. Complement activation: IgG can activate the complement system, a group of proteins that work together to help eliminate pathogens from the body. Activation of the complement system leads to the formation of the membrane attack complex, which creates holes in the cell membranes of bacteria, leading to their lysis (destruction).
4. Antibody-dependent cellular cytotoxicity (ADCC): IgG can bind to immune cells like natural killer (NK) cells and trigger them to release substances that cause target cells (such as virus-infected or cancerous cells) to undergo apoptosis (programmed cell death).
5. Immune complex formation: IgG can form immune complexes with antigens, which can then be removed from the body through various mechanisms, such as phagocytosis by immune cells or excretion in urine.

IgG is a critical component of adaptive immunity and provides long-lasting protection against reinfection with many pathogens. It has four subclasses (IgG1, IgG2, IgG3, and IgG4) that differ in their structure, function, and distribution in the body.

Tumor Necrosis Factor Ligand Superfamily Member 13 (TNFSF13), also known as APRIL (A Proliferation-Inducing Ligand), is a type II transmembrane protein and a member of the tumor necrosis factor (TNF) ligand superfamily. It plays a crucial role in the immune system, particularly in the activation, proliferation, and differentiation of B cells, which are key players in the humoral immune response.

TNFSF13 is expressed by various cell types, including macrophages, dendritic cells, and neutrophils. It binds to two receptors: TACI (Transmembrane Activator and Calcium Modulator and Cyclophilin Ligand Interactor) and BCMA (B-cell Maturation Antigen), which are primarily found on the surface of B cells. The interaction between TNFSF13 and its receptors promotes the survival, proliferation, and differentiation of B cells into plasma cells, ultimately leading to increased antibody production.

Dysregulation of TNFSF13 has been implicated in several autoimmune and inflammatory diseases, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), and multiple sclerosis (MS). Therefore, targeting this molecule or its signaling pathways has been a focus of research for the development of novel therapeutic strategies in these conditions.

T-lymphocytes, also known as T-cells, are a type of white blood cell that plays a key role in the adaptive immune system's response to infection. They are produced in the bone marrow and mature in the thymus gland. There are several different types of T-cells, including CD4+ helper T-cells, CD8+ cytotoxic T-cells, and regulatory T-cells (Tregs).

CD4+ helper T-cells assist in activating other immune cells, such as B-lymphocytes and macrophages. They also produce cytokines, which are signaling molecules that help coordinate the immune response. CD8+ cytotoxic T-cells directly kill infected cells by releasing toxic substances. Regulatory T-cells help maintain immune tolerance and prevent autoimmune diseases by suppressing the activity of other immune cells.

T-lymphocytes are important in the immune response to viral infections, cancer, and other diseases. Dysfunction or depletion of T-cells can lead to immunodeficiency and increased susceptibility to infections. On the other hand, an overactive T-cell response can contribute to autoimmune diseases and chronic inflammation.

Immunophenotyping is a medical laboratory technique used to identify and classify cells, usually in the context of hematologic (blood) disorders and malignancies (cancers), based on their surface or intracellular expression of various proteins and antigens. This technique utilizes specific antibodies tagged with fluorochromes, which bind to the target antigens on the cell surface or within the cells. The labeled cells are then analyzed using flow cytometry, allowing for the detection and quantification of multiple antigenic markers simultaneously.

Immunophenotyping helps in understanding the distribution of different cell types, their subsets, and activation status, which can be crucial in diagnosing various hematological disorders, immunodeficiencies, and distinguishing between different types of leukemias, lymphomas, and other malignancies. Additionally, it can also be used to monitor the progression of diseases, evaluate the effectiveness of treatments, and detect minimal residual disease (MRD) during follow-up care.

Immunoglobulin A (IgA) is a type of antibody that plays a crucial role in the immune function of the human body. It is primarily found in external secretions, such as saliva, tears, breast milk, and sweat, as well as in mucous membranes lining the respiratory and gastrointestinal tracts. IgA exists in two forms: a monomeric form found in serum and a polymeric form found in secretions.

The primary function of IgA is to provide immune protection at mucosal surfaces, which are exposed to various environmental antigens, such as bacteria, viruses, parasites, and allergens. By doing so, it helps prevent the entry and colonization of pathogens into the body, reducing the risk of infections and inflammation.

IgA functions by binding to antigens present on the surface of pathogens or allergens, forming immune complexes that can neutralize their activity. These complexes are then transported across the epithelial cells lining mucosal surfaces and released into the lumen, where they prevent the adherence and invasion of pathogens.

In summary, Immunoglobulin A (IgA) is a vital antibody that provides immune defense at mucosal surfaces by neutralizing and preventing the entry of harmful antigens into the body.

Opportunistic infections (OIs) are infections that occur more frequently or are more severe in individuals with weakened immune systems, often due to a underlying condition such as HIV/AIDS, cancer, or organ transplantation. These infections are caused by microorganisms that do not normally cause disease in people with healthy immune function, but can take advantage of an opportunity to infect and cause damage when the body's defense mechanisms are compromised. Examples of opportunistic infections include Pneumocystis pneumonia, tuberculosis, candidiasis (thrush), and cytomegalovirus infection. Preventive measures, such as antimicrobial medications and vaccinations, play a crucial role in reducing the risk of opportunistic infections in individuals with weakened immune systems.

HIV-1 (Human Immunodeficiency Virus type 1) is a species of the retrovirus genus that causes acquired immunodeficiency syndrome (AIDS). It is primarily transmitted through sexual contact, exposure to infected blood or blood products, and from mother to child during pregnancy, childbirth, or breastfeeding. HIV-1 infects vital cells in the human immune system, such as CD4+ T cells, macrophages, and dendritic cells, leading to a decline in their numbers and weakening of the immune response over time. This results in the individual becoming susceptible to various opportunistic infections and cancers that ultimately cause death if left untreated. HIV-1 is the most prevalent form of HIV worldwide and has been identified as the causative agent of the global AIDS pandemic.

CD27 is a protein that is found on the surface of certain immune cells, including T cells and B cells. It is a type of molecule known as a cell-surface antigen, which can be recognized by other immune cells and used to target those cells for activation or destruction. CD27 plays a role in the regulation of the immune response, particularly in the activation and differentiation of T cells.

CD27 is also a member of the tumor necrosis factor receptor (TNFR) superfamily, which means that it has a specific structure and function that allows it to interact with other molecules called ligands. The interaction between CD27 and its ligand, CD70, helps to activate T cells and promote their survival and proliferation.

In addition to its role in the immune response, CD27 has also been studied as a potential target for cancer immunotherapy. Because CD27 is expressed on certain types of tumor cells, it may be possible to use therapies that target CD27 to stimulate an immune response against the tumor and help to destroy it. However, more research is needed to determine the safety and effectiveness of these approaches.

Immunologic memory, also known as adaptive immunity, refers to the ability of the immune system to recognize and mount a more rapid and effective response upon subsequent exposure to a pathogen or antigen that it has encountered before. This is a key feature of the vertebrate immune system and allows for long-term protection against infectious diseases.

Immunologic memory is mediated by specialized cells called memory T cells and B cells, which are produced during the initial response to an infection or immunization. These cells persist in the body after the pathogen has been cleared and can quickly respond to future encounters with the same or similar antigens. This rapid response leads to a more effective and efficient elimination of the pathogen, resulting in fewer symptoms and reduced severity of disease.

Immunologic memory is the basis for vaccines, which work by exposing the immune system to a harmless form of a pathogen or its components, inducing an initial response and generating memory cells that provide long-term protection against future infections.

Cardiovascular abnormalities refer to structural or functional anomalies in the heart or blood vessels. These abnormalities can be present at birth (congenital) or acquired later in life. They can affect the heart's chambers, valves, walls, or blood vessels, leading to various complications such as heart failure, stroke, or even death if left untreated.

Examples of congenital cardiovascular abnormalities include:

1. Septal defects - holes in the walls separating the heart's chambers (atrial septal defect, ventricular septal defect)
2. Valvular stenosis or insufficiency - narrowing or leakage of the heart valves
3. Patent ductus arteriosus - a persistent opening between the aorta and pulmonary artery
4. Coarctation of the aorta - narrowing of the aorta
5. Tetralogy of Fallot - a combination of four heart defects, including ventricular septal defect, overriding aorta, pulmonary stenosis, and right ventricular hypertrophy

Examples of acquired cardiovascular abnormalities include:

1. Atherosclerosis - the buildup of plaque in the arteries, leading to narrowing or blockage
2. Cardiomyopathy - disease of the heart muscle, causing it to become enlarged, thickened, or stiffened
3. Hypertension - high blood pressure, which can damage the heart and blood vessels over time
4. Myocardial infarction (heart attack) - damage to the heart muscle due to blocked blood supply
5. Infective endocarditis - infection of the inner lining of the heart chambers and valves

These abnormalities can be diagnosed through various tests, such as echocardiography, electrocardiogram (ECG), stress testing, cardiac catheterization, or magnetic resonance imaging (MRI). Treatment options depend on the type and severity of the abnormality and may include medications, medical procedures, or surgery.

Interleukin-2 (IL-2) is a type of cytokine, which are signaling molecules that mediate and regulate immunity, inflammation, and hematopoiesis. Specifically, IL-2 is a growth factor for T cells, a type of white blood cell that plays a central role in the immune response. It is primarily produced by CD4+ T cells (also known as T helper cells) and stimulates the proliferation and differentiation of activated T cells, including effector T cells and regulatory T cells. IL-2 also has roles in the activation and function of other immune cells, such as B cells, natural killer cells, and dendritic cells. Dysregulation of IL-2 production or signaling can contribute to various pathological conditions, including autoimmune diseases, chronic infections, and cancer.

A lymphocyte count is a laboratory test that measures the number of white blood cells called lymphocytes in a sample of blood. Lymphocytes are a vital part of the immune system and help fight off infections and diseases. A normal lymphocyte count ranges from 1,000 to 4,800 cells per microliter (µL) of blood for adults.

An abnormal lymphocyte count can indicate an infection, immune disorder, or blood cancer. A low lymphocyte count is called lymphopenia, while a high lymphocyte count is called lymphocytosis. The cause of an abnormal lymphocyte count should be investigated through further testing and clinical evaluation.

Hemolytic anemia, autoimmune is a type of anemia characterized by the premature destruction of red blood cells (RBCs) in which the immune system mistakenly attacks and destroys its own RBCs. This occurs when the body produces autoantibodies that bind to the surface of RBCs, leading to their rupture (hemolysis). The symptoms may include fatigue, weakness, shortness of breath, and dark colored urine. The diagnosis is made through blood tests that measure the number and size of RBCs, reticulocyte count, and the presence of autoantibodies. Treatment typically involves suppressing the immune system with medications such as corticosteroids or immunosuppressive drugs, and sometimes removal of the spleen (splenectomy) may be necessary.

Interstitial lung diseases (ILDs) are a group of disorders characterized by inflammation and scarring (fibrosis) in the interstitium, the tissue and space around the air sacs (alveoli) of the lungs. The interstitium is where the blood vessels that deliver oxygen to the lungs are located. ILDs can be caused by a variety of factors, including environmental exposures, medications, connective tissue diseases, and autoimmune disorders.

The scarring and inflammation in ILDs can make it difficult for the lungs to expand and contract normally, leading to symptoms such as shortness of breath, cough, and fatigue. The scarring can also make it harder for oxygen to move from the air sacs into the bloodstream.

There are many different types of ILDs, including:

* Idiopathic pulmonary fibrosis (IPF): a type of ILD that is caused by unknown factors and tends to progress rapidly
* Hypersensitivity pneumonitis: an ILD that is caused by an allergic reaction to inhaled substances, such as mold or bird droppings
* Connective tissue diseases: ILDs can be a complication of conditions such as rheumatoid arthritis and scleroderma
* Sarcoidosis: an inflammatory disorder that can affect multiple organs, including the lungs
* Asbestosis: an ILD caused by exposure to asbestos fibers

Treatment for ILDs depends on the specific type of disease and its underlying cause. Some treatments may include corticosteroids, immunosuppressive medications, and oxygen therapy. In some cases, a lung transplant may be necessary.

Acquired Immunodeficiency Syndrome (AIDS) is a chronic, life-threatening condition caused by the Human Immunodeficiency Virus (HIV). AIDS is the most advanced stage of HIV infection, characterized by the significant weakening of the immune system, making the person more susceptible to various opportunistic infections and cancers.

The medical definition of AIDS includes specific criteria based on CD4+ T-cell count or the presence of certain opportunistic infections and diseases. According to the Centers for Disease Control and Prevention (CDC), a person with HIV is diagnosed with AIDS when:

1. The CD4+ T-cell count falls below 200 cells per cubic millimeter of blood (mm3) - a normal range is typically between 500 and 1,600 cells/mm3.
2. They develop one or more opportunistic infections or cancers that are indicative of advanced HIV disease, regardless of their CD4+ T-cell count.

Some examples of these opportunistic infections and cancers include:

* Pneumocystis pneumonia (PCP)
* Candidiasis (thrush) affecting the esophagus, trachea, or lungs
* Cryptococcal meningitis
* Toxoplasmosis of the brain
* Cytomegalovirus disease
* Kaposi's sarcoma
* Non-Hodgkin's lymphoma
* Invasive cervical cancer

It is important to note that with appropriate antiretroviral therapy (ART), people living with HIV can maintain their CD4+ T-cell counts, suppress viral replication, and prevent the progression to AIDS. Early diagnosis and consistent treatment are crucial for managing HIV and improving life expectancy and quality of life.

Simian Immunodeficiency Virus (SIV) is a retrovirus that primarily infects African non-human primates and is the direct ancestor of Human Immunodeficiency Virus type 2 (HIV-2). It is similar to HIV in its structure, replication strategy, and ability to cause an immunodeficiency disease in its host. SIV infection in its natural hosts is typically asymptomatic and non-lethal, but it can cause AIDS-like symptoms in other primate species. Research on SIV in its natural hosts has provided valuable insights into the mechanisms of HIV pathogenesis and potential strategies for prevention and treatment of AIDS.

The CD4-CD8 ratio is a measurement of the relative numbers of two types of immune cells, CD4+ T cells (also known as helper T cells) and CD8+ T cells (also known as cytotoxic T cells), in the blood. The CD4-CD8 ratio is commonly used as a marker of immune function and health.

CD4+ T cells play an important role in the immune response by helping to coordinate the activity of other immune cells, producing chemical signals that activate them, and producing antibodies. CD8+ T cells are responsible for directly killing infected cells and tumor cells.

A normal CD4-CD8 ratio is typically between 1.0 and 3.0. A lower ratio may indicate an impaired immune system, such as in cases of HIV infection or other immunodeficiency disorders. A higher ratio may be seen in some viral infections, autoimmune diseases, or cancer. It's important to note that the CD4-CD8 ratio should be interpreted in conjunction with other laboratory and clinical findings for a more accurate assessment of immune function.

Autoimmunity is a medical condition in which the body's immune system mistakenly attacks and destroys healthy tissues within the body. In normal function, the immune system recognizes and fights off foreign substances such as bacteria, viruses, and toxins. However, when autoimmunity occurs, the immune system identifies self-molecules or tissues as foreign and produces an immune response against them.

This misguided response can lead to chronic inflammation, tissue damage, and impaired organ function. Autoimmune diseases can affect various parts of the body, including the joints, skin, glands, muscles, and blood vessels. Some common examples of autoimmune diseases are rheumatoid arthritis, lupus, multiple sclerosis, type 1 diabetes, Hashimoto's thyroiditis, and Graves' disease.

The exact cause of autoimmunity is not fully understood, but it is believed to involve a combination of genetic, environmental, and lifestyle factors that trigger an abnormal immune response in susceptible individuals. Treatment for autoimmune diseases typically involves managing symptoms, reducing inflammation, and suppressing the immune system's overactive response using medications such as corticosteroids, immunosuppressants, and biologics.

A splenectomy is a surgical procedure in which the spleen is removed from the body. The spleen is an organ located in the upper left quadrant of the abdomen, near the stomach and behind the ribs. It plays several important roles in the body, including fighting certain types of infections, removing old or damaged red blood cells from the circulation, and storing platelets and white blood cells.

There are several reasons why a splenectomy may be necessary, including:

* Trauma to the spleen that cannot be repaired
* Certain types of cancer, such as Hodgkin's lymphoma or non-Hodgkin's lymphoma
* Sickle cell disease, which can cause the spleen to enlarge and become damaged
* A ruptured spleen, which can be life-threatening if not treated promptly
* Certain blood disorders, such as idiopathic thrombocytopenic purpura (ITP) or hemolytic anemia

A splenectomy is typically performed under general anesthesia and may be done using open surgery or laparoscopically. After the spleen is removed, the incision(s) are closed with sutures or staples. Recovery time varies depending on the individual and the type of surgery performed, but most people are able to return to their normal activities within a few weeks.

It's important to note that following a splenectomy, individuals may be at increased risk for certain types of infections, so it's recommended that they receive vaccinations to help protect against these infections. They should also seek medical attention promptly if they develop fever, chills, or other signs of infection.

CD4-positive T-lymphocytes, also known as CD4+ T cells or helper T cells, are a type of white blood cell that plays a crucial role in the immune response. They express the CD4 receptor on their surface and help coordinate the immune system's response to infectious agents such as viruses and bacteria.

CD4+ T cells recognize and bind to specific antigens presented by antigen-presenting cells, such as dendritic cells or macrophages. Once activated, they can differentiate into various subsets of effector cells, including Th1, Th2, Th17, and Treg cells, each with distinct functions in the immune response.

CD4+ T cells are particularly important in the immune response to HIV (human immunodeficiency virus), which targets and destroys these cells, leading to a weakened immune system and increased susceptibility to opportunistic infections. The number of CD4+ T cells is often used as a marker of disease progression in HIV infection, with lower counts indicating more advanced disease.

Infection is defined medically as the invasion and multiplication of pathogenic microorganisms such as bacteria, viruses, fungi, or parasites within the body, which can lead to tissue damage, illness, and disease. This process often triggers an immune response from the host's body in an attempt to eliminate the infectious agents and restore homeostasis. Infections can be transmitted through various routes, including airborne particles, direct contact with contaminated surfaces or bodily fluids, sexual contact, or vector-borne transmission. The severity of an infection may range from mild and self-limiting to severe and life-threatening, depending on factors such as the type and quantity of pathogen, the host's immune status, and any underlying health conditions.

Antibody formation, also known as humoral immune response, is the process by which the immune system produces proteins called antibodies in response to the presence of a foreign substance (antigen) in the body. This process involves several steps:

1. Recognition: The antigen is recognized and bound by a type of white blood cell called a B lymphocyte or B cell, which then becomes activated.
2. Differentiation: The activated B cell undergoes differentiation to become a plasma cell, which is a type of cell that produces and secretes large amounts of antibodies.
3. Antibody production: The plasma cells produce and release antibodies, which are proteins made up of four polypeptide chains (two heavy chains and two light chains) arranged in a Y-shape. Each antibody has two binding sites that can recognize and bind to specific regions on the antigen called epitopes.
4. Neutralization or elimination: The antibodies bind to the antigens, neutralizing them or marking them for destruction by other immune cells. This helps to prevent the spread of infection and protect the body from harmful substances.

Antibody formation is an important part of the adaptive immune response, which allows the body to specifically recognize and respond to a wide variety of pathogens and foreign substances.

CD (cluster of differentiation) antigens are cell-surface proteins that are expressed on leukocytes (white blood cells) and can be used to identify and distinguish different subsets of these cells. They are important markers in the field of immunology and hematology, and are commonly used to diagnose and monitor various diseases, including cancer, autoimmune disorders, and infectious diseases.

CD antigens are designated by numbers, such as CD4, CD8, CD19, etc., which refer to specific proteins found on the surface of different types of leukocytes. For example, CD4 is a protein found on the surface of helper T cells, while CD8 is found on cytotoxic T cells.

CD antigens can be used as targets for immunotherapy, such as monoclonal antibody therapy, in which antibodies are designed to bind to specific CD antigens and trigger an immune response against cancer cells or infected cells. They can also be used as markers to monitor the effectiveness of treatments and to detect minimal residual disease (MRD) after treatment.

It's important to note that not all CD antigens are exclusive to leukocytes, some can be found on other cell types as well, and their expression can vary depending on the activation state or differentiation stage of the cells.

T-lymphocyte subsets refer to distinct populations of T-cells, which are a type of white blood cell that plays a central role in cell-mediated immunity. The two main types of T-lymphocytes are CD4+ and CD8+ cells, which are defined by the presence or absence of specific proteins called cluster differentiation (CD) molecules on their surface.

CD4+ T-cells, also known as helper T-cells, play a crucial role in activating other immune cells, such as B-lymphocytes and macrophages, to mount an immune response against pathogens. They also produce cytokines that help regulate the immune response.

CD8+ T-cells, also known as cytotoxic T-cells, directly kill infected cells or tumor cells by releasing toxic substances such as perforins and granzymes.

The balance between these two subsets of T-cells is critical for maintaining immune homeostasis and mounting effective immune responses against pathogens while avoiding excessive inflammation and autoimmunity. Therefore, the measurement of T-lymphocyte subsets is essential in diagnosing and monitoring various immunological disorders, including HIV infection, cancer, and autoimmune diseases.

Flow cytometry is a medical and research technique used to measure physical and chemical characteristics of cells or particles, one cell at a time, as they flow in a fluid stream through a beam of light. The properties measured include:

* Cell size (light scatter)
* Cell internal complexity (granularity, also light scatter)
* Presence or absence of specific proteins or other molecules on the cell surface or inside the cell (using fluorescent antibodies or other fluorescent probes)

The technique is widely used in cell counting, cell sorting, protein engineering, biomarker discovery and monitoring disease progression, particularly in hematology, immunology, and cancer research.

Respiratory tract infections (RTIs) are infections that affect the respiratory system, which includes the nose, throat (pharynx), voice box (larynx), windpipe (trachea), bronchi, and lungs. These infections can be caused by viruses, bacteria, or, less commonly, fungi.

RTIs are classified into two categories based on their location: upper respiratory tract infections (URTIs) and lower respiratory tract infections (LRTIs). URTIs include infections of the nose, sinuses, throat, and larynx, such as the common cold, flu, laryngitis, and sinusitis. LRTIs involve the lower airways, including the bronchi and lungs, and can be more severe. Examples of LRTIs are pneumonia, bronchitis, and bronchiolitis.

Symptoms of RTIs depend on the location and cause of the infection but may include cough, congestion, runny nose, sore throat, difficulty breathing, wheezing, fever, fatigue, and chest pain. Treatment for RTIs varies depending on the severity and underlying cause of the infection. For viral infections, treatment typically involves supportive care to manage symptoms, while antibiotics may be prescribed for bacterial infections.

Lymphoproliferative disorders (LPDs) are a group of diseases characterized by the excessive proliferation of lymphoid cells, which are crucial components of the immune system. These disorders can arise from both B-cells and T-cells, leading to various clinical manifestations ranging from benign to malignant conditions.

LPDs can be broadly classified into reactive and neoplastic categories:

1. Reactive Lymphoproliferative Disorders: These are typically triggered by infections, autoimmune diseases, or immunodeficiency states. They involve an exaggerated response of the immune system leading to the excessive proliferation of lymphoid cells. Examples include:
* Infectious mononucleosis (IM) caused by Epstein-Barr virus (EBV)
* Lymph node enlargement due to various infections or autoimmune disorders
* Post-transplant lymphoproliferative disorder (PTLD), which occurs in the context of immunosuppression following organ transplantation
2. Neoplastic Lymphoproliferative Disorders: These are malignant conditions characterized by uncontrolled growth and accumulation of abnormal lymphoid cells, leading to the formation of tumors. They can be further classified into Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Examples include:
* Hodgkin lymphoma (HL): Classical HL and nodular lymphocyte-predominant HL
* Non-Hodgkin lymphoma (NHL): Various subtypes, such as diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, and Burkitt lymphoma

It is important to note that the distinction between reactive and neoplastic LPDs can sometimes be challenging, requiring careful clinical, histopathological, immunophenotypic, and molecular evaluations. Proper diagnosis and classification of LPDs are crucial for determining appropriate treatment strategies and predicting patient outcomes.

Lymphocyte subsets refer to distinct populations of white blood cells called lymphocytes, which are crucial components of the adaptive immune system. There are two main types of lymphocytes: T cells and B cells, and each type has several subsets based on their surface receptors, functions, and activation status.

1. T cell subsets: These include CD4+ T helper cells (Th cells), CD8+ cytotoxic T cells (Tc cells), regulatory T cells (Tregs), and memory T cells. Th cells are further divided into Th1, Th2, Th17, and Tfh cells based on their cytokine production profiles and functions.
* CD4+ T helper cells (Th cells) play a central role in orchestrating the immune response by producing various cytokines that activate other immune cells.
* CD8+ cytotoxic T cells (Tc cells) directly kill virus-infected or malignant cells upon recognition of specific antigens presented on their surface.
* Regulatory T cells (Tregs) suppress the activation and proliferation of other immune cells to maintain self-tolerance and prevent autoimmunity.
* Memory T cells are long-lived cells that remain in the body after an initial infection or immunization, providing rapid protection upon subsequent encounters with the same pathogen.
2. B cell subsets: These include naïve B cells, memory B cells, and plasma cells. Upon activation by antigens, B cells differentiate into antibody-secreting plasma cells that produce specific antibodies to neutralize or eliminate pathogens.
* Naïve B cells are resting cells that have not yet encountered their specific antigen.
* Memory B cells are long-lived cells generated after initial antigen exposure, which can quickly differentiate into antibody-secreting plasma cells upon re-exposure to the same antigen.
* Plasma cells are terminally differentiated B cells that secrete large amounts of specific antibodies.

Analyzing lymphocyte subsets is essential for understanding immune system function and dysfunction, as well as monitoring the effectiveness of immunotherapies and vaccinations.

HIV (Human Immunodeficiency Virus) infection is a viral illness that progressively attacks and weakens the immune system, making individuals more susceptible to other infections and diseases. The virus primarily infects CD4+ T cells, a type of white blood cell essential for fighting off infections. Over time, as the number of these immune cells declines, the body becomes increasingly vulnerable to opportunistic infections and cancers.

HIV infection has three stages:

1. Acute HIV infection: This is the initial stage that occurs within 2-4 weeks after exposure to the virus. During this period, individuals may experience flu-like symptoms such as fever, fatigue, rash, swollen glands, and muscle aches. The virus replicates rapidly, and the viral load in the body is very high.
2. Chronic HIV infection (Clinical latency): This stage follows the acute infection and can last several years if left untreated. Although individuals may not show any symptoms during this phase, the virus continues to replicate at low levels, and the immune system gradually weakens. The viral load remains relatively stable, but the number of CD4+ T cells declines over time.
3. AIDS (Acquired Immunodeficiency Syndrome): This is the most advanced stage of HIV infection, characterized by a severely damaged immune system and numerous opportunistic infections or cancers. At this stage, the CD4+ T cell count drops below 200 cells/mm3 of blood.

It's important to note that with proper antiretroviral therapy (ART), individuals with HIV infection can effectively manage the virus, maintain a healthy immune system, and significantly reduce the risk of transmission to others. Early diagnosis and treatment are crucial for improving long-term health outcomes and reducing the spread of HIV.

The hygiene hypothesis is not a formally recognized medical definition, but rather a term used to describe a proposed explanation for the increase in allergic and autoimmune diseases in developed countries. The hypothesis suggests that early childhood exposure to microorganisms and certain infectious agents can help mature and regulate the immune system, reducing the risk of developing these conditions later in life.

In modern, highly sanitized environments, children may not be exposed to as many microbes during their formative years, leading to an overactive immune response that can manifest as allergies or autoimmune disorders. It's important to note that this is a theoretical concept and there is ongoing research to better understand the relationship between early-life exposures, the immune system, and the development of allergic and autoimmune diseases.

An encyclopedia is a comprehensive reference work containing articles on various topics, usually arranged in alphabetical order. In the context of medicine, a medical encyclopedia is a collection of articles that provide information about a wide range of medical topics, including diseases and conditions, treatments, tests, procedures, and anatomy and physiology. Medical encyclopedias may be published in print or electronic formats and are often used as a starting point for researching medical topics. They can provide reliable and accurate information on medical subjects, making them useful resources for healthcare professionals, students, and patients alike. Some well-known examples of medical encyclopedias include the Merck Manual and the Stedman's Medical Dictionary.

Autoimmune diseases are a group of disorders in which the immune system, which normally protects the body from foreign invaders like bacteria and viruses, mistakenly attacks the body's own cells and tissues. This results in inflammation and damage to various organs and tissues in the body.

In autoimmune diseases, the body produces autoantibodies that target its own proteins or cell receptors, leading to their destruction or malfunction. The exact cause of autoimmune diseases is not fully understood, but it is believed that a combination of genetic and environmental factors contribute to their development.

There are over 80 different types of autoimmune diseases, including rheumatoid arthritis, lupus, multiple sclerosis, type 1 diabetes, Hashimoto's thyroiditis, Graves' disease, psoriasis, and inflammatory bowel disease. Symptoms can vary widely depending on the specific autoimmune disease and the organs or tissues affected. Treatment typically involves managing symptoms and suppressing the immune system to prevent further damage.

Communicable diseases, also known as infectious diseases, are illnesses that can be transmitted from one person to another through various modes of transmission. These modes include:

1. Direct contact: This occurs when an individual comes into physical contact with an infected person, such as touching or shaking hands, or having sexual contact.
2. Indirect contact: This happens when an individual comes into contact with contaminated objects or surfaces, like doorknobs, towels, or utensils.
3. Airborne transmission: Infectious agents can be spread through the air when an infected person coughs, sneezes, talks, or sings, releasing droplets containing the pathogen into the environment. These droplets can then be inhaled by nearby individuals.
4. Droplet transmission: Similar to airborne transmission, but involving larger respiratory droplets that don't remain suspended in the air for long periods and typically travel shorter distances (usually less than 6 feet).
5. Vector-borne transmission: This occurs when an infected animal or insect, such as a mosquito or tick, transmits the disease to a human through a bite or other means.

Examples of communicable diseases include COVID-19, influenza, tuberculosis, measles, hepatitis B, and malaria. Preventive measures for communicable diseases often involve public health initiatives like vaccination programs, hygiene promotion, and vector control strategies.

Hygiene is the science and practice of maintaining and promoting health and preventing disease through cleanliness in personal and public environments. It includes various measures such as handwashing, bathing, using clean clothes, cleaning and disinfecting surfaces, proper waste disposal, safe food handling, and managing water supplies to prevent the spread of infectious agents like bacteria, viruses, and parasites.

In a medical context, hygiene is crucial in healthcare settings to prevent healthcare-associated infections (HAIs) and ensure patient safety. Healthcare professionals are trained in infection control practices, including proper hand hygiene, use of personal protective equipment (PPE), environmental cleaning and disinfection, and safe injection practices.

Overall, maintaining good hygiene is essential for overall health and well-being, reducing the risk of illness and promoting a healthy lifestyle.

Emerging communicable diseases are infections whose incidence has increased in the past two decades or threatens to increase in the near future. These diseases can be caused by new microbial agents, or by previously known agents that have newly acquired the ability to cause disease in humans. They may also result from changes in human demographics, behavior, or travel patterns, or from technological or environmental changes. Examples of emerging communicable diseases include COVID-19, Ebola virus disease, Zika virus infection, and West Nile fever.

The two most common cancers in patients with common variable immunodeficiency include lymphoma and certain stomach cancers. The ... People with common variable immunodeficiency have trouble fighting off infections due to the lack of antibodies produced, which ... Common variable immunodeficiency (CVID) is an immune disorder characterized by recurrent infections and low antibody levels, ... risk for these given cancers is almost fifty times greater in patients with common variable immunodeficiency than in those ...
... common variable immunodeficiency; severe combined immunodeficiency; immunoglobulin-G-subclass deficiencies with recurrent ... although some small-scale studies have indicated that a particular cohort of patients with common variable immunodeficiency ( ... Immunoglobulin therapy is also used in some treatment protocols for secondary immunodeficiencies such as human immunodeficiency ... Common side effects include pain at the site of injection, muscle pain, and allergic reactions. Other severe side effects ...
Furthermore, those with Common Variable Immunodeficiency (CVID) may be at even higher risk. One study of 80 CVID patients found ... "Sarcoidosis and common variable immunodeficiency. Report of 8 cases and review of the literature". Medicine. 75 (5): 251-261. ... The most common abnormality is involvement of the facial nerve, which may lead to reduced power on one or both sides of the ... The variable incidence of neurosarcoidosis over the world may be at least partially attributable to the lack of screening ...
IgA deficiency and common variable immunodeficiency (CVID) feature similar B cell differentiation arrests, but it does not ... 2002). "Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a combined ... Hammarström, L.; Vorechovsky, I.; Webster, D. (2000). "Selective IgA deficiency (SIgAD) and common variable immunodeficiency ( ... and common variable immunodeficiency (CVID)". Clinical and Experimental Immunology. 120 (2): 225-231. doi:10.1046/j.1365- ...
It has been suggested that Good Syndrome is a subset of common variable immunodeficiency (CVID). The mainstay of treatment ... The immunodeficiency may occur before or after the diagnosis of a thymoma. Immunodeficiency involves both deficient humoral and ... Thymoma with immunodeficiency (also known as "Good syndrome") is a rare disorder that occurs in adults in whom ... Immunodeficiency does not resolve after thymectomy. Immunosuppression is sometimes used. The Centers for Disease Control and ...
Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency. J Allergy Clin Immunol 2004;114: ... Granulomatous-lymphocytic interstitial lung disease (GLILD) is a lung complication of common variable immunodeficiency ... Multicenter experience in hematopoietic stem cell transplantation for serious complications of common variable immunodeficiency ... in patients with common variable immunodeficiency (CVID). J Clin Immunol 2013;33:30-39 PMID 22930256 PMC3557581 Wehr C, Gennery ...
"Respiratory Infections and Antibiotic Usage in Common Variable Immunodeficiency" (PDF). The Journal of Allergy and Clinical ... Respiratory Infections and Antibiotic Usage in Common Variable Immunodeficiency Epidemiology, Pathogenesis and Treatment of ... He is currently the Principal Investigator of the STILLPAD-UK study, a prospective study of lung disease in Immunodeficiency. ... the largest Primary Immunodeficiency Centre in Europe, Seneviratne was a Consultant and Lead Clinician in Clinical Immunology ...
"TACI is mutant in common variable immunodeficiency and IgA deficiency". Nature Genetics. 37 (8): 829-34. doi:10.1038/ng1601. ... TACI mutations are associated with immunodeficiency in humans, as a significant proportion of CVID patients have TACI mutations ...
Unravelling the complexity of T cell abnormalities in common variable immunodeficiency. The Journal of Immunology, 178(6), 3932 ... "Family and linkage study of selective IgA deficiency and common variable immunodeficiency". Clinical Immunology and ... 2005 study linked positive family history to a higher risk for immunoglobulin A deficiency and common variable immunodeficiency ... He also served on the Immunodeficiency Committee at the World Allergy Organization. Paganelli's research has focused on the ...
October 2004). "Common variable immunodeficiency is associated with defective functions of dendritic cells". Blood. 104 (8): ... exemplified by low dendritic cell counts resulting in common variable immunodeficiency (CVID). For example, gut cells secrete ...
"Homozygous loss of ICOS is associated with adult-onset common variable immunodeficiency". Nature Immunology. 4 (3): 261-8. doi: ...
"common variable immunodeficiency" at Dorland's Medical Dictionary Barmettler, Sara (14 September 2021). "Hypogammaglobulinemia ... such as common variable immunodeficiency, or it may be caused by secondary effects such as medication, blood cancer, or poor ... common variable immunodeficiency (CVID), hyper-IgM syndromes, IgG subclass deficiency, isolated non-IgG immunoglobulin ... CVID is the most common form of primary immunodeficiency. SCID is considered a medical emergency and suspected cases require ...
Antibody deficiencies are also common, including common variable immunodeficiency and IgA deficiency. T-cell immunity is normal ... The most common characteristics include a distinct craniofacial phenotype (microcephaly, micrognathia, short philtrum, ... Less common characteristics include hypospadias, colobomata of the iris, renal anomalies, and deafness. ... Commons category link from Wikidata, Autosomal monosomies and deletions, Rare syndromes, Genodermatoses). ...
"Disseminated Mycoplasma orale infection in a patient with common variable immunodeficiency syndrome". Diagnostic Microbiology ... Contamination is common due to their small physical size, lack of cell wall, and pleomorphism. Pleomorphism is defined as an ... This comes as no surprise, as the class Mollicutes contains common commensals or pathogens of several different organisms. Many ...
"Primary cutaneous actinomycosis caused by Actinomyces bovis in a patient with common variable immunodeficiency". The Journal of ... Common causes of trauma to the oral mucosa include consumption of abrasive feed or foreign bodies (thorns, wire, etc.), or ... The other explains its common name, lumpy jaw, as A. bovis bacteria infect the mandibular bone, causing osteomyelitis and the ...
"Optimal use of ivig in a patient with Behçet syndrome and common variable immunodeficiency". Ann. Allergy Asthma Immunol. 109 ( ... The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation ... While rare in the United States and Europe, it is more common in the Middle East and Asia. In Turkey, for example, about 2 per ... Most common arterial lesions are occlusions or stenosis and aneurysms or pseudoaneurysms.[citation needed] The cause is not ...
Hypogammaglobulinemia Common variable immunodeficiency (CVID) Hyper-IgM syndromes Barton JC, Bertoli LF, Acton RT (June 2003 ... "HLA-A and -B alleles and haplotypes in 240 index patients with common variable immunodeficiency and selective IgG subclass ... IgG subclass deficiencies are also an integral component of other well-known primary immunodeficiency diseases, such as Wiskott ... IgG4 subclass deficiency is very common, but mostly completely asymptomatic. Since the formation of antibodies is a complicated ...
1999) and Baldwin (1996).[supplied by OMIM] Mutation of the NFKB2 gene has been linked to Common variable immunodeficiency ( ... "Germline mutations in NFKB2 implicate the noncanonical NF-κB pathway in the pathogenesis of common variable immunodeficiency". ... "NFKB2 mutation in common variable immunodeficiency and isolated adrenocorticotropic hormone deficiency: A case report and ... The mutation occurs at the C-terminus of NFKB2 and it causes common variable immunodeficienciency which in turn causes ...
"Individuals with IgA deficiency and common variable immunodeficiency share polymorphisms of major histocompatibility complex ... whereas HLA types more common in Italy, Greece and the middle east are more common within this region of France. Other ... People with many common ancestors from Ireland share similar risks of disease. In the case of juvenile diabetes a clear ... A common serologically defined haplotype in Europeans is HLA A1-B8-DR3-DQ2.5 (see above). In non-persistent sarcoidosis this ...
"Symptomatic hypoglycemia in a child with common variable immunodeficiency: Deficient anterior pituitary with variable immune ... genetic disorder that is characterized by adrenocorticotropic hormone deficiency combined with common variable immunodeficiency ... DAVID syndrome, short for deficient anterior pituitary with variable immune deficiency syndrome, is a rare ... having variable endocrine and immune deficiencies". BMC Medical Genetics. 15: 139. doi:10.1186/s12881-014-0139-9. PMC 4411703. ...
X-linked agammaglobulinemia Common variable immunodeficiency (CVID) "X-linked Immunodeficiency With Hyper IgM Clinical ... mutation due to skewed X-chromosome inactivation can present with symptoms resembling HIGM1 or common variable immunodeficiency ... The fungus is common and is present in over 70% of healthy people's lungs, however, Hyper IgM patients are not able to fight it ... The most common causes are mutations in the CD40 Ligand (CD40LG) gene located at Xq26.3-27 leading to X-linked HIGM (XHIGM) in ...
Common variable immunodeficiency is thought to be due to a problem in the differentiation from lymphocytes to plasma cells. The ... and do not express common pan-B cell markers, such as CD19 and CD20. Instead, plasma cells are identified through flow ...
Children with common variable immunodeficiency (CVID) are also at a higher risk of developing a lymphoproliferative disorder.[ ... Viral infection is a very common cause of lymphoproliferative disorders. In children, the most common is believed to be ... The Epstein-Barr virus, which infects >90% of the world population, is also a common cause of these disorders, being ... Boys with X-linked immunodeficiency syndrome are at a higher risk of mortality associated with Epstein-Barr virus infections, ...
Additionally, conditions like common variable immunodeficiency (CVID), severe combined immunodeficiency (SCID), and certain ... These disorders are common in immunocompromised individuals and involve abnormal proliferation of T and B cells, often ... Wikimedia Commons has media related to Lymphocytes. Histology image: 01701ooa - Histology Learning System at Boston University ... One basis for low T cell lymphocytes occurs when the human immunodeficiency virus (HIV) infects and destroys T cells ( ...
Certain other immune deficiency states (e.g. common variable immunodeficiency and IgA deficiency) are also associated with ... December 2002). "Cancer risk among patients with IgA deficiency or common variable immunodeficiency and their relatives: a ... Because chronic viral hepatitis is so common, and liver cancer so deadly, liver cancer is one of the most common causes of ... Worldwide in 2015, the most common causes of cancer death were lung cancer (1.6 million deaths), liver cancer (745,000 deaths ...
J. C. van Meurs, S. Lightman, P. W. T. De Waard et al., "Retinal vasculitis occurring with common variable immunodeficiency ... Behçet's disease Common Variable Immune Deficiency Eales disease Granulomatosis with polyangiitis Idiopathic Retinal Vasculitis ...
2002). "Analysis of SWAP-70 as a candidate gene for non-X-linked hyper IgM syndrome and common variable immunodeficiency". Clin ...
... an oral IL-12/IL-23 inhibitor for the treatment of autoimmune diseases and common variable immunodeficiency". IDrugs. 10 (1): ...
Common variable immunodeficiency IPEX syndrome Along with treatment for infections and other complications several additional ... The presentation of this condition is variable making the diagnosis difficult. The most common features include Immune ...
This includes, but is not limited to, common variable immunodeficiency, X-linked agammaglobulinemia, congenital ... Common applications are ophthalmic surgery, in combination with local anesthetics. It also increases the absorption rate of ... The resulting hyaluronic acid fragments of variable size are then further hydrolyzed by HYAL1 after being internalized into ... ISBN 978-0-8153-4105-5. Media related to Hyaluronidase at Wikimedia Commons "Rituximab and Hyaluronidase Human Injection". ...
The two most common cancers in patients with common variable immunodeficiency include lymphoma and certain stomach cancers. The ... People with common variable immunodeficiency have trouble fighting off infections due to the lack of antibodies produced, which ... Common variable immunodeficiency (CVID) is an immune disorder characterized by recurrent infections and low antibody levels, ... risk for these given cancers is almost fifty times greater in patients with common variable immunodeficiency than in those ...
Common variable immunodeficiency (CVID) is a disorder that involves the following: (1) low levels of most or all of the ... encoded search term (Common Variable Immunodeficiency) and Common Variable Immunodeficiency What to Read Next on Medscape ... Common variable immunodeficiency (CVID), the most common clinical primary immunodeficiency in adults, is a disorder that ... In patients with common variable immunodeficiency (CVID), numerous immune-system abnormalities are reported, the most common of ...
New insights into common variable immunodeficiency. Ann Intern Med. 1993;118:720-30.PubMedGoogle Scholar ... West Nile Virus Meningitis in Patient with Common Variable Immunodeficiency. Emerging Infectious Diseases. 2003;9(10):1353-1354 ... West Nile Virus Meningitis in Patient with Common Variable Immunodeficiency On This Page ... West Nile Virus Meningitis in Patient with Common Variable Immunodeficiency. Volume 9, Number 10-October 2003 ...
002008 - Impact of Asthma on Hospitalization and Antibiotic Courses in Common Variable Immunodeficiency Respiratory Infections ... Impact of Asthma on Hospitalization and Antibiotic Courses in Common Variable Immunodeficiency Respiratory Infections ... 2024/sessions/e-tps-display-only/impact-of-asthma-on-hospitalization-and-antibiotic-courses-in-common-variable-immunodeficiency ...
Common variable immunodeficiency (CVID) is an innate immunity error, possibly associated with recurrent or chronic infections ... diagnosed with common variable immunodeficiency, according to the criteria of the European Immunodeficiency Society [14], were ... Common Variable Immunodeficiency (CVID) is a syndrome encompassing a heterogeneous group of diseases, appearing at any age, ... Background: Common variable immunodeficiency (CVID) is an innate immunity error, possibly associated with recurrent or chronic ...
The common immunologic defect in patients with common variable immunodeficiency is defective antibody formation. As is expected ... encoded search term (Pediatric Common Variable Immunodeficiency) and Pediatric Common Variable Immunodeficiency What to Read ... Pediatric Common Variable Immunodeficiency Medication. Updated: Apr 25, 2014 * Author: C Lucy Park, MD; Chief Editor: Harumi ... Cellular abnormalities in common variable immunodeficiency. In: Rosen FS, Seligmann M, eds. Immunodeficiencies. Philadelphia: ...
Do you qualify for these Common Variable Immunodeficiency studies? Were researching treatments for 2024. ... Abatacept for the Treatment of Common Variable Immunodeficiency With Interstitial Lung Disease open to eligible people ages 4 ... Common Variable Immunodeficiency clinical trials at UCSF 1 research study open to eligible people ... Our lead scientists for Common Variable Immunodeficiency research studies include Michele Pham, MD. ...
... severe combined immunodeficiency disorder (SCID), and common variable immunodeficiency disorder. ... Immunodeficiency-associated vaccine-derived polioviruses (iVDPVs) emerge among persons with primary immunodeficiencies (PIDs) ... primary immunodeficiency disorder; SCID = severe combined immunodeficiency disorder.. * Data as of May 17, 2020.. † Percentage ... Immunodeficiency-associated VDPVs (iVDPVs) are isolated from persons with primary immunodeficiency (PID). Infection with iVDPV ...
How CVID affects the immune system Common variable immunodeficiency (CVID) is a genetic disorder that causes people to develop ... Common Variable Immunodeficiency (CVID) Education. How CVID affects the immune system. Common variable immunodeficiency (CVID) ... What is common variable immunodeficiency (CVID)?. CVID is classified as a primary immunodeficiency disease (PIDD). These ... What are the common symptoms of CVID?. CVID symptoms vary widely from person to person. They can range from mild to severe. ...
Common variable immunodeficiency is a heterogeneous group of diseases of unknown etiology. Patients have low serum ...
Common Variable Immunodeficiency (CVID) - Learn about the causes, symptoms, diagnosis & treatment from the MSD Manuals - ... Common variable immunodeficiency is a primary immunodeficiency disorder Primary immunodeficiency Immunodeficiency disorders ... Common variable immunodeficiency is an immunodeficiency disorder characterized by very low antibody (immunoglobulin) levels ... Common Variable Immunodeficiency (CVID) By James Fernandez , MD, PhD, Cleveland Clinic Lerner College of Medicine at Case ...
Common variable immune deficiency (CVID) is a disorder that impairs the immune system. Explore symptoms, inheritance, genetics ... Genetics of common variable immunodeficiency: role of transmembrane activator and calcium modulator and cyclophilin ligand ... medlineplus.gov/genetics/condition/common-variable-immune-deficiency/ Common variable immune deficiency. ... IMMUNODEFICIENCY, COMMON VARIABLE, 3; CVID3. *IMMUNODEFICIENCY, COMMON VARIABLE, 4; CVID4. *IMMUNODEFICIENCY, COMMON VARIABLE, ...
Common Variable Immunodeficiency / enzymology * Common Variable Immunodeficiency / genetics* * Common Variable Immunodeficiency ...
Common variable immunodeficiency.. *Graft-vs-host disease.. Our Pathologists. *Sarangarajan Ranganathan, MD - Division Director ...
... of patients with common variable immunodeficiency (CVID), and occasionally in patients with other primary immunodeficiency ... Tumor necrosis factor and intravenous gammaglobulins in common variable immunodeficiency. Clin Immunol Immunopathol (1994) 72: ... Thobani S, Hu E, Huynh P, Scott L. Common variable immunodeficiency: a patient with anaphylaxis to intravenous and subcutaneous ... Anti-IgA antibodies in common variable immunodeficiency (CVID): diagnostic workup and therapeutic strategy. Clin Immunol (2007 ...
Phenotypic and functional assays were used to profile CMV-specific T cells in patients with common variable immunodeficiency ... CMV-specific immune response in the debilitating chronic inflammatory complications of common variable immunodeficiency. ... Common variable immunodeficiency is the most common primary immunodeficiency. A subset of patients has debilitating ... BACKGROUND: Common variable immunodeficiency is the most common primary immunodeficiency. A subset of patients has debilitating ...
Direct and indirect costs of immunoglobulin replacement therapy in patients with common variable immunodeficiency (CVID) and X- ... Direct and indirect costs of immunoglobulin replacement therapy in patients with common variable immunodeficiency (CVID) and X- ...
Common variable immunodeficiency. *Chronic granulomatous disease of childhood. Primary Ciliary Dyskinesia *Kartageners ... Asthenopia is a common term for eye strain, or discomfort with prolonged use. It is not damaging to... ... Clubbing of the fingers, which is not usually seen in other types of obstructive lung disease, is more common in moderate to ...
Antibody deficiencies such as common variable immunodeficiency (CVID) and X-linked agammaglobulinaemia. ... ASCIA PC FAST FACTS Primary Immunodeficiencies 202376.81 KB. * Primary immunodeficiency (PID) disorders, also known as inborn ... Combined immunodeficiencies such as severe combined immunodeficiency (SCID).. *Phagocytic cell deficiencies such as chronic ... IEI/PID disorders are different to AIDS (acquired immunodeficiency syndrome), that is due to human immunodeficiency virus (HIV ...
Next Generation Sequencing to Diagnose Primary Immunodeficiency - Genomics and Precision Health Blog ... The study included participants with common variable immunodeficiency (CVID), which is rarely monogenic. After excluding these ... Categories genomics, primary immunodeficiency. Tags Next Generation Sequencing. Post a Comment. Cancel Reply. Your email ... Primary immunodeficiencies (PI) are a group of more than 400 genetic disorders that alter the ability of the immune system to ...
Common variable immunodeficiency and idiopathic primary hypogammaglobulinemia: two different conditions within the same disease ... Expression of CD8 is associated with non-common type morphology and outcome in pediatric anaplastic lymphoma kinase-positive ...
Primary immunodeficiency diseases (e.g., common variable immune deficiency and IgA deficiency) ... primary immunodeficiencies (IgA deficiency and common variable immune deficiency), and hematopoietic stem cell and cord blood ... eRA Commons - Applicants must have an active DUNS number to register in eRA Commons. Organizations can register with the eRA ... Failure to register in the Commons and to include a valid PD/PI Commons ID in the credential field will prevent the successful ...
Predictive markers for humoral influenza vaccine response in patients with common variable immunodeficiency. ... Bioavailability of gamma-globulin after subcutaneous infusions in patients with common variable immunodeficiency. ... Journal article: IMMUNODEFICIENCY. 1993;4(1-4):81-84 Safety of rapid subcutaneous gammaglobulin infusions in patients with ... Evaluation of ColdZyme® Mouth Spray on prevention of upper respiratory tract infections in a boy with primary immunodeficiency ...
Common variable immunodeficiency as the initial presentation of dyskeratosis congenita. J Allergy Clin Immunol. 2013 Jul. 132(1 ... Therapeutic interventions are mostly palliative, but BMT and SCT for aplastic anemia have been tried with variable success. ... A recessive founder mutation in regulator of telomere elongation helicase 1, RTEL1, underlies severe immunodeficiency and ... Dyskeratosis congenita: a combined immunodeficiency with broad clinical spectrum--a single-center pediatric experience. Pediatr ...
Studies of Immune Regulation in Patients with Common Variable Immunodeficiency and Related Humoral Immunodeficiency Syndromes. ... Molecular and Clinical Studies of Primary Immunodeficiency Diseases. No longer recruiting/follow-up only. 3-125 Years. NHGRI. ... Pilot Trial of Allogeneic Blood or Marrow Transplantation for Primary Immunodeficiencies. Participants currently recruited/ ...
This includes, but is not limited to, the humoral immune defect in common variable immunodeficiency (CVID), X-linked ... There were 51 subjects (81%) with common variable immunodeficiency as their primary diagnosis, followed by X-linked ... 1.1 Primary Humoral Immunodeficiency 2 DOSAGE AND ADMINISTRATION 2.1 Preparation and Handling 2.2 Recommended Dose 2.3 ... 1 Treatment of Primary Humoral Immunodeficiency 15 REFERENCES 16 HOW SUPPLIED/STORAGE AND HANDLING 17 PATIENT COUNSELING ...
... common variable immunodeficiency (CVID), X-linked agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined ... 1.1 Primary Humoral Immunodeficiency. PRIVIGEN is indicated as replacement therapy for primary humoral immunodeficiency (PI). ... Primary Immunodeficiency. 200-800 mg/kg (2-8 mL/kg). every 3-4 weeks. 0.5 mg/kg/min. (0.005 mL/kg/min). Increase to 8 mg/kg/min ... HIV-1, human immunodeficiency virus type 1, a model for HIV-1 and HIV-2; PRV, pseudorabies virus, a nonspecific model for large ...
One example is common variable immunodeficiency (CVID) where multiple autoimmune diseases are seen, e.g.: inflammatory bowel ... Immunodeficiency and autoimmunity edit There are a large number of immunodeficiency syndromes that present clinical and ... Many common human autoimmune diseases can be seen to have a substantial innate immune mediated immunopathology using this new ... The first two, which are involved in the recognition of antigens, are inherently variable and susceptible to recombination. ...
... in whom common variable immunodeficiency syndrome (CVID) previously had been diagnosed. This report summarizes the clinical and ... including persons infected with human immunodeficiency virus. Based on an overall review of available data, the TCG concluded ... the paralytic manifestations were the event that prompted consideration of the diagnosis of immunodeficiency. There is no ... in this report is exceptional because it is the only known VAPP case in an immunodeficient person in which immunodeficiency had ...
  • Common variable immunodeficiency (CVID) is an immune disorder characterized by recurrent infections and low antibody levels, specifically in immunoglobulin (Ig) types IgG, IgM, and IgA. (wikipedia.org)
  • Common infections include: Pneumonia Ear infections Sinusitis Chronic coughing (lasting from a few weeks to many months) Gastrointestinal infections Gastrovascular infections or inflammation are very common for those with CVID. (wikipedia.org)
  • Lymphoma is a common cause of death in patients with CVID. (medscape.com)
  • Common variable immunodeficiency (CVID), the most common clinical primary immunodeficiency in adults, is a disorder that involves the following: (1) low levels of most or all of the immunoglobulin (Ig) classes, (2) a lack of B lymphocytes or plasma cells that are capable of producing antibodies, and (3) frequent bacterial infections. (medscape.com)
  • In September 2002, during a WNV epidemic in Michigan ( 2 ), a 38-year-old woman with common variable immunodeficiency (CVID) sought treatment at the University of Michigan Hospital with acute WNV-associated meningitis. (cdc.gov)
  • Common variable immunodeficiency (CVID) is an innate immunity error, possibly associated with recurrent or chronic infections and autoimmune / inflammatory diseases and neoplasms. (researchsquare.com)
  • There is no standard of care therapy for patients with granulomatous-lymphocytic interstitial lung disease (GLILD) seen in common variable immunodeficiency (CVID). (ucsf.edu)
  • Common variable immunodeficiency (CVID) is a genetic disorder that causes people to develop frequent infections. (promptcare.com)
  • What is common variable immunodeficiency (CVID)? (promptcare.com)
  • CVID is classified as a primary immunodeficiency disease (PIDD). (promptcare.com)
  • What are the common symptoms of CVID? (promptcare.com)
  • Common variable immune deficiency (CVID) is a disorder that impairs the immune system. (medlineplus.gov)
  • Pneumonia is common in people with CVID. (medlineplus.gov)
  • Antibody deficiencies such as common variable immunodeficiency (CVID) and X-linked agammaglobulinaemia. (allergy.org.au)
  • The study included participants with common variable immunodeficiency (CVID), which is rarely monogenic. (cdc.gov)
  • Recently completed molecular studies of poliovirus isolates suggest that viral replication of vaccine-related polioviruses may have persisted for as long as 7 years in a patient with vaccine-associated paralytic poliomyelitis (VAPP) in whom common variable immunodeficiency syndrome (CVID) previously had been diagnosed. (cdc.gov)
  • Since the introduction of IgGs, survival rates of patients with common variable immune deficiency (CVID) have increased from 30% in 1979 to an almost normal life expectancy for patients without disease-related complications. (euneedsmoreplasma.com)
  • In 2016, patients of both genders aged from 9 to 61 years, diagnosed with common variable immunodeficiency, according to the criteria of the European Immunodeficiency Society [ 14 ], were evaluated through a controlled cross-sectional study, conducted in the Discipline of Allergy, Clinical Immunology and Pediatric Rheumatology, Federal University of São Paulo - São Paulo Medicine School (UNIFESP-EPM). (researchsquare.com)
  • I enjoy teaching very much and I have been educating students and staff for 30 years about clinical immunology, primary immunodeficiencies and care of patients with these conditions. (ki.se)
  • People with common variable immunodeficiency may have a chronic cough, cough up blood, or have difficulty breathing (due to frequent sinus and lung infections) and may have diarrhea. (msdmanuals.com)
  • CONCLUSION: Our data strongly support a direct role for CMV and a hyperreactive CMV-specific immune response in the debilitating chronic inflammatory complications of common variable immunodeficiency. (ox.ac.uk)
  • BIVIGAM is an Immune Globulin Intravenous (Human), 10% Liquid, indicated for the treatment of adults and pediatric patients 2 years of age and older with primary humoral immunodeficiency (PI). (nih.gov)
  • Overview of Immunodeficiency Disorders Immunodeficiency disorders involve malfunction of the immune system, resulting in infections that develop and recur more frequently, are more severe, and last longer than usual. (msdmanuals.com)
  • Primary immunodeficiency (PID) disorders, also known as inborn errors of immunity (IEI) are a group of more than 400 potentially serious disorders that can lead to frequent or severe infections, swellings, and autoimmune problems. (allergy.org.au)
  • IEI/PID disorders are different to AIDS (acquired immunodeficiency syndrome), that is due to human immunodeficiency virus (HIV). (allergy.org.au)
  • Primary immunodeficiencies (PI) are a group of more than 400 genetic disorders that alter the ability of the immune system to fight off infection and affect 1 out of 1,200 1 births in the United States. (cdc.gov)
  • Common variable immunodeficiency disorders: division into distinct clinical phenotypes. (euneedsmoreplasma.com)
  • Immunodeficiency-related lymphoproliferative disorders (LPDs) form a heterogeneous group that ranges from self-limiting hyperplasias to aggressive lymphomas. (medscape.com)
  • Variable" refers to the heterogeneous clinical manifestations of this disorder, which include recurrent bacterial infections, increased risk for autoimmune disease and lymphoma, as well as gastrointestinal disease. (wikipedia.org)
  • The two most common cancers in patients with common variable immunodeficiency include lymphoma and certain stomach cancers. (wikipedia.org)
  • An excess of lymphoma in specific PIDD populations principally drove this increased incidence, while no increased risk of the most common solid tumor malignancies was observed. (northwestern.edu)
  • Common variable immunodeficiency is a heterogeneous group of diseases of unknown etiology. (lu.se)
  • As with any antibody deficiency, the most common types of infections and illnesses involve the ears, sinuses, nose, and lungs. (wikipedia.org)
  • People with common variable immunodeficiency have trouble fighting off infections due to the lack of antibodies produced, which normally resist invading microbes. (wikipedia.org)
  • Patients with agammaglobulinemia, either common variable or X-linked, are known to be susceptible to recurrent infections ( 3 ). (cdc.gov)
  • Patients with more profound immunodeficiency or patients with active infections have more severe reactions. (medscape.com)
  • Evaluation of ColdZyme® Mouth Spray on prevention of upper respiratory tract infections in a boy with primary immunodeficiency: a case report. (ki.se)
  • Immunodeficiency-associated vaccine-derived polioviruses (iVDPVs) emerge among persons with primary immunodeficiencies (PIDs) and rarely can persist. (cdc.gov)
  • Background: We evaluated the overall and site-specific incidence of cancer in subjects with primary immunodeficiency diseases (PIDD) enrolled in the United States Immune Deficiency Network (USIDNET) registry compared with age-adjusted cancer incidence in the Surveillance, Epidemiology and End Results Program (SEER) database. (northwestern.edu)
  • Of the 4 most common malignancies in men and women in SEER (lung, colon, breast, and prostate cancers), we found no significant increase in these diagnoses in subjects with PIDD. (northwestern.edu)
  • Homozygous autosomal recessive mutations in RTEL1 lead to similar phenotypes that parallel with Hoyeraal-Hreidarsson (HH) syndrome, a severe variant of DKC characterized by cerebellar hypoplasia, bone marrow failure, intrauterine growth restriction and immunodeficiency. (medscape.com)
  • The iVDPV case definition is a laboratory-confirmed VDPV infection in a person of any age who has a primary humoral (B-cell) or combined humoral and cellular (B- and T-cell) immunodeficiency disorder ( 6 ). (cdc.gov)
  • 8. Positive serological tests for hepatitis B, hepatitis C (also confirmed with HCV RNA), or human immunodeficiency virus (HIV) infection, or known infection status at Visit 2. (who.int)
  • Common variable immunodeficiency is an immunodeficiency disorder characterized by very low antibody (immunoglobulin) levels despite a normal number of B cells (the type of white blood cell that makes antibodies). (msdmanuals.com)
  • Combined immunodeficiencies such as severe combined immunodeficiency (SCID). (allergy.org.au)
  • The case described in this report is exceptional because it is the only known VAPP case in an immunodeficient person in which immunodeficiency had been diagnosed before onset of paralytic manifestations (5). (cdc.gov)
  • I am one of the founders of the International 'Nursing Group for Immunodeficiencies' (https://ingid.org). (ki.se)
  • Introduction Immunoglobulin represents the main therapy for patients with inborn errors of immunity (IEI) and it is a safe procedure, but adverse events (AEs) can occur with variable frequencies. (bvsalud.org)
  • Pneumonia (5/5) and mycobacterial disease (3/5) were the most common initial manifestations. (bvsalud.org)
  • Inflammation in common variable immunodeficiency is associated with a distinct CD8(+) response to cytomegalovirus. (ox.ac.uk)
  • Skin reactions (rash or urticaria) were the most common AE with 14 episodes (0.8% of all infusions). (bvsalud.org)
  • Novel Common Genetic Susceptibility Loci for Colorectal Cancer. (uksh.de)
  • Ulcerative colitis in a patient with common variable immunodeficiency: does the treatment differ from the routine? (bvsalud.org)
  • The risk for these given cancers is almost fifty times greater in patients with common variable immunodeficiency than in those without. (wikipedia.org)
  • METHODS: Phenotypic and functional assays were used to profile CMV-specific T cells in patients with common variable immunodeficiency with and without inflammatory complications. (ox.ac.uk)
  • Predictive markers for humoral influenza vaccine response in patients with common variable immunodeficiency. (ki.se)
  • COVID-19 vaccination in patients with primary immunodeficiencies: an international survey on patient vaccine hesitancy and self-reported adverse events. (ki.se)
  • The 2015 Workshop on Immunodeficiency and Dysregulation, organized by the Society for Hematopathology (SH) and the European Association for Haematopathology (EAHP), brought together a large number of well-annotated cases from diverse clinical backgrounds and included 415 cases from 21 countries. (medscape.com)
  • The genetic mutations that cause common variable immunodeficiency can be inherited, but more often, they occur spontaneously. (msdmanuals.com)
  • Pneumonia is one of the most common causes of death worldwide. (msdmanuals.com)
  • These cases offered the opportunity to evaluate and compare a wide spectrum of immunodeficiency-related LPDs, assess progress in the field, build on the current understanding and approach to classification, and explore areas for future study. (medscape.com)
  • BACKGROUND: Common variable immunodeficiency is the most common primary immunodeficiency. (ox.ac.uk)