An extranodal neoplasm, usually possessing an NK-cell phenotype and associated with EPSTEIN-BARR VIRUS. These lymphomas exhibit a broad morphologic spectrum, frequent necrosis, angioinvasion, and most commonly present in the midfacial region, but also in other extranodal sites.
Tumors or cancer of the NOSE.
A group of heterogeneous lymphoid tumors representing malignant transformations of T-lymphocytes.
A general term for various neoplastic diseases of the lymphoid tissue.
Bone marrow-derived lymphocytes that possess cytotoxic properties, classically directed against transformed and virus-infected cells. Unlike T CELLS; and B CELLS; NK CELLS are not antigen specific. The cytotoxicity of natural killer cells is determined by the collective signaling of an array of inhibitory and stimulatory CELL SURFACE RECEPTORS. A subset of T-LYMPHOCYTES referred to as NATURAL KILLER T CELLS shares some of the properties of this cell type.
A group of heterogeneous lymphoid tumors generally expressing one or more B-cell antigens or representing malignant transformations of B-lymphocytes.
Any of a group of malignant tumors of lymphoid tissue that differ from HODGKIN DISEASE, being more heterogeneous with respect to malignant cell lineage, clinical course, prognosis, and therapy. The only common feature among these tumors is the absence of giant REED-STERNBERG CELLS, a characteristic of Hodgkin's disease.
A group of malignant lymphomas thought to derive from peripheral T-lymphocytes in lymph nodes and other nonlymphoid sites. They include a broad spectrum of lymphocyte morphology, but in all instances express T-cell markers admixed with epithelioid histiocytes, plasma cells, and eosinophils. Although markedly similar to large-cell immunoblastic lymphoma (LYMPHOMA, LARGE-CELL, IMMUNOBLASTIC), this group's unique features warrant separate treatment.
Malignant lymphoma composed of large B lymphoid cells whose nuclear size can exceed normal macrophage nuclei, or more than twice the size of a normal lymphocyte. The pattern is predominantly diffuse. Most of these lymphomas represent the malignant counterpart of B-lymphocytes at midstage in the process of differentiation.
Extranodal lymphoma of lymphoid tissue associated with mucosa that is in contact with exogenous antigens. Many of the sites of these lymphomas, such as the stomach, salivary gland, and thyroid, are normally devoid of lymphoid tissue. They acquire mucosa-associated lymphoid tissue (MALT) type as a result of an immunologically mediated disorder.
Infection with human herpesvirus 4 (HERPESVIRUS 4, HUMAN); which may facilitate the development of various lymphoproliferative disorders. These include BURKITT LYMPHOMA (African type), INFECTIOUS MONONUCLEOSIS, and oral hairy leukoplakia (LEUKOPLAKIA, HAIRY).
A hydrolase enzyme that converts L-asparagine and water to L-aspartate and NH3. EC 3.5.1.1.
Glycoproteins expressed on cortical thymocytes and on some dendritic cells and B-cells. Their structure is similar to that of MHC Class I and their function has been postulated as similar also. CD1 antigens are highly specific markers for human LANGERHANS CELLS.
The type species of LYMPHOCRYPTOVIRUS, subfamily GAMMAHERPESVIRINAE, infecting B-cells in humans. It is thought to be the causative agent of INFECTIOUS MONONUCLEOSIS and is strongly associated with oral hairy leukoplakia (LEUKOPLAKIA, HAIRY;), BURKITT LYMPHOMA; and other malignancies.
Cerebrosides which contain as their polar head group a galactose moiety bound in glycosidic linkage to the hydroxyl group of ceramide. Their accumulation in tissue, due to a defect in beta-galactosidase, is the cause of galactosylceramide lipidosis or globoid cell leukodystrophy.
Malignant lymphoma in which the lymphomatous cells are clustered into identifiable nodules within the LYMPH NODES. The nodules resemble to some extent the GERMINAL CENTER of lymph node follicles and most likely represent neoplastic proliferation of lymph node-derived follicular center B-LYMPHOCYTES.
A subclass of NK cell lectin-like receptors that includes both inhibitory and stimulatory members.
A classification of T-lymphocytes, especially into helper/inducer, suppressor/effector, and cytotoxic subsets, based on structurally or functionally different populations of cells.
A form of undifferentiated malignant LYMPHOMA usually found in central Africa, but also reported in other parts of the world. It is commonly manifested as a large osteolytic lesion in the jaw or as an abdominal mass. B-cell antigens are expressed on the immature cells that make up the tumor in virtually all cases of Burkitt lymphoma. The Epstein-Barr virus (HERPESVIRUS 4, HUMAN) has been isolated from Burkitt lymphoma cases in Africa and it is implicated as the causative agent in these cases; however, most non-African cases are EBV-negative.
DNA sequences encoding the alpha chain of the T-cell receptor. The genomic organization of the TcR alpha genes is essentially the same in all species and is similar to the organization of Ig genes.
A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.
A major histocompatibily complex class I-like protein that plays a unique role in the presentation of lipid ANTIGENS to NATURAL KILLER T-CELLS.
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.

EBV-associated, extranodal NK-cell lymphoma, nasal type of the breast, after heart transplantation. (1/85)

Post-transplantation lymphoproliferative disorders (PTLDs) are predominantly Epstein-Barr virus (EBV)-associated B-cell lymphoproliferations. PTLDs of T-cell lineage are rare, mostly reported after renal transplantation and show less frequent association with EBV. NK-cell lymphomas after transplantation (NK-cell PTLDs) are very rare; only five cases are reported so far in the English literature, all developed after renal transplantation. We describe a case of EBV-associated, extranodal NK-cell lymphoma of nasal type, involving the breast in a cardiac allograft recipient 5 years after transplantation. The neoplastic cells are positive for CD2, cytoplasmic CD3, CD7, CD43, CD56, TIA-1 and p53; and negative for surface CD3 and CD57. Analysis of T-cell receptor beta and gamma genes fails to show clonal rearrangement. EBV studies show clonal episomal integration of EBV and latency II pattern (EBER-1+, LMP-1+, EBNA-1+, EBNA-2-). In conclusion, NK-cell PTLDs are rare complications that arise relatively late after solid organ transplantation, show strong association with EBV, and can follow an aggressive clinical course. To the best of our knowledge, we present the first reported case of NK-cell PTLD after cardiac transplantation and the unifying clinical and diagnostic features of NK-cell PTLDs occurring after solid organ transplantation.  (+info)

Ki-67 expression is predictive of prognosis in patients with stage I/II extranodal NK/T-cell lymphoma, nasal type. (2/85)

BACKGROUND: Localized extranodal natural killer (NK)/T-cell lymphoma, nasal type, commonly has a low or low-intermediate risk of the international prognostic index (IPI), so the IPI has shown inconsistency in predicting prognosis. Thus, we analyzed Ki-67 expression and proposed a new prognostic model including Ki-67 expression for stage I/II extranodal NK/T-cell lymphoma. PATIENTS AND METHODS: We studied Ki-67 expression and its relationship with prognosis in 50 patients with extranodal NK/T-cell lymphoma. RESULTS: The patients were dichotomized by the median value: low (<65%) versus high Ki-67 (> or =65%). High Ki-67 was associated with a worse overall survival (OS; P = 0.021) and disease-free survival (DFS; P = 0.044). In multivariate analysis, Ki-67 expression and primary site of involvement were found to be an independent prognostic factor for OS and DFS (P < 0.05). Based on these results, we proposed a new clinico-pathological prognostic model with Ki-67 expression and the primary site of involvement. It showed a high degree of correlation with worse OS and DFS (P < 0.001). CONCLUSIONS: Ki-67 expression is predictive of prognosis, and our prognostic model may become a useful tool for predicting prognosis in patients with stage I/II extranodal NK/T-cell lymphoma, nasal type.  (+info)

Mediastinal single nodal relapse of a nasal NK/T cell lymphoma. (3/85)

A nasal NK/T cell lymphoma is a very aggressive form of lymphoma. Patterns of relapse after treatment have not been systematically evaluated, and mediastinal nodal relapse at a primary site has never been documented. We describe here a 40-year old man who presented with a nasal obstruction caused by a protruding mass that was identified as a nasal NK/T cell lymphoma. The initial work-up, including chest and abdominopelvic computed tomography (CT) and positron emission tomography (PET), showed no regional or distant metastasis. A CT scan performed following three cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) showed that the mass had nearly disappeared. Radiation therapy undertaken following chemotherapy was given to the primary site. However, PET performed following radiotherapy revealed a single mediastinal lymphadenopathy, with no evidence of residual tumor in the nasal cavity. A biopsy using video-assisted thoracoscopy (VATS) showed the presence of a recurrent NK/T cell lymphoma with an immunophenotype identical to that of the primary nasal lymphoma. An additional three cycles of CHOP chemotherapy were administered, and the patient remains alive, with no evidence of disease 30 months after the initial relapse. These findings indicate that early detection with PET and prompt surgical excision with the use of VATS can lead to successful treatment of a relapsed nasal NK/T cell lymphoma.  (+info)

Nasal-type NK/T-cell lymphoma: a case report. (4/85)

Extranodal NK/T-cell lymphoma represents less than 1% of all lymphomas, but is more common in Asia and South America. We present a 67-year-old female with a 10-month history of four reddish-blue firm and painful nodules in the parietal region of the head, ranging in size from 1 to 5 cm. Two nodules were taken for biopsy, which showed atypical lymphoid cells with angiocentric growth pattern. The immunophenotype of the tumor cells was CD45RO +, CD56 +, CD3 + (epsilon chain), CD20-, consistent with the diagnosis of NK/T-cell lymphoma. NK/T-cell lymphomas are rare and the optimal treatment has not been clearly established.  (+info)

The enhanced expression of the matrix metalloproteinase 9 in nasal NK/T-cell lymphoma. (5/85)

BACKGROUND: Nasal NK/T cell lymphoma is an aggressive disease and has a poor prognosis. Nasal NK/T cell lymphoma is refractory to conventional chemotherapy and has strong tendency of widespread relapse or dissemination into distant sites. METHODS: We immunohistochemically studied nasal NK/T-cell lymphoma to elucidate the unique characteristics of nasal NK/T-cell lymphoma, such as its higher metastatic tendency and its vast necrosis which leads to destruction of the involved tissues. The expression of P-glycoprotein and MMP-9 was evaluated in the 20 patients with nasal NK/T-cell lymphoma and 25 with nasal non-NK/T-cell lymphoma and the relationship between expression of these proteins and clinical results were analyzed in this report. RESULTS: Overall 5-year survival rates for patients with nasal NK/T cell lymphoma, and nasal non-NK/T cell lymphoma were 51%, and 84%. Distant involvement free 5-year survival rates for patients with nasal NK/T cell lymphoma, and nasal non-NK/T cell lymphoma were 53%, and 79%. Overall positivity for P-glycoprotein was observed in 10 of 19 patients with NTL and in 13 of 23 patients with non-NTL. When the overall survival rate was compared between patients with P-glycoprotein positive and negative, there was no difference between them. Sixteen of the 19 patients with nasal NK/T cell lymphoma expressed MMP-9. In contrast, only 8 of the 22 patients with nasal non-NK/T cell lymphoma expressed MMP-9. Distant involvement free 5-year survival rates for patients with MMP-9 negative, and MMP-9 positive were 92%, and 61%, respectively. The difference was statistically significant (p = 0.027). CONCLUSION: Positive immunoreactivity for P-glycoprotein was not an independent prognostic factor in nasal NK/T-cell lymphomas, which stresses the importance of exploring other mechanisms of drug resistance. The strong expression of MMP-9 is uniquely characteristic of nasal NK/T cell lymphoma and may contribute to its strong tendency to disseminatate and the extensive necrosis which is always seen. However, our results are based on univariate comparisons, and as such, should be viewed with some caution.  (+info)

Clinical heterogeneity of extranodal NK/T-cell lymphoma, nasal type: a national survey of the Korean Cancer Study Group. (6/85)

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Overexpression of Syk tyrosine kinase in peripheral T-cell lymphomas. (7/85)

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Effective second-line chemotherapy for extranodal NK/T-cell lymphoma consisting of etoposide, ifosfamide, methotrexate, and prednisolone. (8/85)

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Extranodal NK-T-cell lymphoma is a rare and aggressive type of lymphoma that typically involves the nasal area and other extranodal sites. It is characterized by the proliferation of natural killer (NK) cells or T-cells, specifically those that express the CD56 surface antigen and are positive for cytoplasmic CD3 epsilon.

The tumor cells in this type of lymphoma often produce large amounts of cytokines, leading to extensive tissue destruction and necrosis at the site of involvement. The disease can also involve the skin, gastrointestinal tract, lungs, and other organs.

Extranodal NK-T-cell lymphoma is more prevalent in Asians and Latin Americans than in other populations. It tends to affect middle-aged adults and has a poor prognosis, with a high rate of relapse and a low survival rate. Treatment typically involves a combination of chemotherapy, radiation therapy, and sometimes stem cell transplantation.

Nose neoplasms refer to abnormal growths or tumors in the nasal cavity or paranasal sinuses. These growths can be benign (non-cancerous) or malignant (cancerous). Benign neoplasms are typically slow-growing and do not spread to other parts of the body, while malignant neoplasms can invade surrounding tissues and have the potential to metastasize.

Nose neoplasms can cause various symptoms such as nasal congestion, nosebleeds, difficulty breathing through the nose, loss of smell, facial pain or numbness, and visual changes if they affect the eye. The diagnosis of nose neoplasms usually involves a combination of physical examination, imaging studies (such as CT or MRI scans), and biopsy to determine the type and extent of the growth. Treatment options depend on the type, size, location, and stage of the neoplasm and may include surgery, radiation therapy, chemotherapy, or a combination of these approaches.

T-cell lymphoma is a type of cancer that affects the T-cells, which are a specific type of white blood cell responsible for immune function. These lymphomas develop from mature T-cells and can be classified into various subtypes based on their clinical and pathological features.

T-cell lymphomas can arise in many different organs, including the lymph nodes, skin, and other soft tissues. They often present with symptoms such as enlarged lymph nodes, fever, night sweats, and weight loss. The diagnosis of T-cell lymphoma typically involves a biopsy of the affected tissue, followed by immunophenotyping and genetic analysis to determine the specific subtype.

Treatment for T-cell lymphomas may include chemotherapy, radiation therapy, immunotherapy, or stem cell transplantation, depending on the stage and aggressiveness of the disease. The prognosis for T-cell lymphoma varies widely depending on the subtype and individual patient factors.

Lymphoma is a type of cancer that originates from the white blood cells called lymphocytes, which are part of the immune system. These cells are found in various parts of the body such as the lymph nodes, spleen, bone marrow, and other organs. Lymphoma can be classified into two main types: Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL).

HL is characterized by the presence of a specific type of abnormal lymphocyte called Reed-Sternberg cells, while NHL includes a diverse group of lymphomas that lack these cells. The symptoms of lymphoma may include swollen lymph nodes, fever, night sweats, weight loss, and fatigue.

The exact cause of lymphoma is not known, but it is believed to result from genetic mutations in the lymphocytes that lead to uncontrolled cell growth and division. Exposure to certain viruses, chemicals, and radiation may increase the risk of developing lymphoma. Treatment options for lymphoma depend on various factors such as the type and stage of the disease, age, and overall health of the patient. Common treatments include chemotherapy, radiation therapy, immunotherapy, and stem cell transplantation.

Natural Killer (NK) cells are a type of lymphocyte, which are large granular innate immune cells that play a crucial role in the host's defense against viral infections and malignant transformations. They do not require prior sensitization to target and destroy abnormal cells, such as virus-infected cells or tumor cells. NK cells recognize their targets through an array of germline-encoded activating and inhibitory receptors that detect the alterations in the cell surface molecules of potential targets. Upon activation, NK cells release cytotoxic granules containing perforins and granzymes to induce target cell apoptosis, and they also produce a variety of cytokines and chemokines to modulate immune responses. Overall, natural killer cells serve as a critical component of the innate immune system, providing rapid and effective responses against infected or malignant cells.

B-cell lymphoma is a type of cancer that originates from the B-lymphocytes, which are a part of the immune system and play a crucial role in fighting infections. These cells can develop mutations in their DNA, leading to uncontrolled growth and division, resulting in the formation of a tumor.

B-cell lymphomas can be classified into two main categories: Hodgkin's lymphoma and non-Hodgkin's lymphoma. B-cell lymphomas are further divided into subtypes based on their specific characteristics, such as the appearance of the cells under a microscope, the genetic changes present in the cancer cells, and the aggressiveness of the disease.

Some common types of B-cell lymphomas include diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, and Burkitt lymphoma. Treatment options for B-cell lymphomas depend on the specific subtype, stage of the disease, and other individual factors. Treatment may include chemotherapy, radiation therapy, immunotherapy, targeted therapy, or stem cell transplantation.

Non-Hodgkin lymphoma (NHL) is a type of cancer that originates in the lymphatic system, which is part of the immune system. It involves the abnormal growth and proliferation of malignant lymphocytes (a type of white blood cell), leading to the formation of tumors in lymph nodes, spleen, bone marrow, or other organs. NHL can be further classified into various subtypes based on the specific type of lymphocyte involved and its characteristics.

The symptoms of Non-Hodgkin lymphoma may include:

* Painless swelling of lymph nodes in the neck, armpits, or groin
* Persistent fatigue
* Unexplained weight loss
* Fever
* Night sweats
* Itchy skin

The exact cause of Non-Hodgkin lymphoma is not well understood, but it has been associated with certain risk factors such as age (most common in people over 60), exposure to certain chemicals, immune system deficiencies, and infection with viruses like Epstein-Barr virus or HIV.

Treatment for Non-Hodgkin lymphoma depends on the stage and subtype of the disease, as well as the patient's overall health. Treatment options may include chemotherapy, radiation therapy, immunotherapy, targeted therapy, stem cell transplantation, or a combination of these approaches. Regular follow-up care is essential to monitor the progression of the disease and manage any potential long-term side effects of treatment.

T-cell peripheral lymphoma is a type of cancer that affects the T-cells, which are a type of white blood cell that plays a crucial role in the body's immune system. This type of lymphoma is called "peripheral" because it typically develops in T-cells that have matured and are found in various tissues and organs outside of the bone marrow, such as the lymph nodes, spleen, skin, and digestive tract.

Peripheral T-cell lymphomas (PTCL) are relatively rare and can be aggressive, with a tendency to spread quickly throughout the body. They can arise from different types of T-cells, leading to various subtypes of PTCL that may have different clinical features, treatment options, and prognoses.

Some common subtypes of peripheral T-cell lymphoma include:

1. PTCL, not otherwise specified (NOS): This is the most common subtype, accounting for about 25-30% of all PTCL cases. It includes cases that do not fit into any specific category or have features of more than one subtype.
2. Anaplastic large cell lymphoma (ALCL): ALCL can be further divided into two groups: systemic ALCL and cutaneous ALCL. Systemic ALCL is a more aggressive form, while cutaneous ALCL tends to be less aggressive and primarily affects the skin.
3. Angioimmunoblastic T-cell lymphoma (AITL): AITL is an aggressive subtype that often involves the lymph nodes and can affect other organs such as the spleen, liver, and bone marrow. It frequently presents with B symptoms (fever, night sweats, and weight loss) and abnormal blood tests.
4. Enteropathy-associated T-cell lymphoma (EATL): EATL is a rare but aggressive subtype that primarily affects the intestines, particularly in individuals with a history of celiac disease or gluten sensitivity.
5. Adult T-cell leukemia/lymphoma (ATLL): ATLL is caused by the human T-cell leukemia virus type 1 (HTLV-1) and primarily affects adults from regions where HTLV-1 is endemic, such as Japan, the Caribbean, and parts of Africa.

Treatment for PTCL depends on the specific subtype, stage, and individual patient factors. Common treatment options include chemotherapy, targeted therapy, immunotherapy, radiation therapy, stem cell transplantation, or a combination of these approaches. Clinical trials are also available for eligible patients to test new therapies and combinations.

Large B-cell lymphoma, diffuse is a type of cancer that starts in cells called B-lymphocytes, which are part of the body's immune system. "Large B-cell" refers to the size and appearance of the abnormal cells when viewed under a microscope. "Diffuse" means that the abnormal cells are spread throughout the lymph node or tissue where the cancer has started, rather than being clustered in one area.

This type of lymphoma is typically aggressive, which means it grows and spreads quickly. It can occur almost anywhere in the body, but most commonly affects the lymph nodes, spleen, and bone marrow. Symptoms may include swollen lymph nodes, fever, night sweats, weight loss, and fatigue.

Treatment for large B-cell lymphoma, diffuse typically involves chemotherapy, radiation therapy, or a combination of both. In some cases, stem cell transplantation or targeted therapy may also be recommended. The prognosis varies depending on several factors, including the stage and location of the cancer, as well as the patient's age and overall health.

B-cell marginal zone lymphoma (MZL) is a type of indolent (slow-growing) non-Hodgkin lymphoma (NHL). It arises from B-lymphocytes, a type of white blood cell found in the lymphatic system. MZLs typically involve the marginal zone of lymphoid follicles, which are structures found in lymph nodes and other lymphatic tissues.

There are three subtypes of MZL: extranodal MZL (also known as mucosa-associated lymphoid tissue or MALT lymphoma), nodal MZL, and splenic MZL. Extranodal MZL is the most common form and can occur at various extranodal sites, such as the stomach, lungs, skin, eyes, and salivary glands. Nodal MZL involves the lymph nodes without evidence of extranodal disease, while splenic MZL primarily affects the spleen.

MZLs are typically low-grade malignancies, but they can transform into more aggressive forms over time. Treatment options depend on the stage and location of the disease, as well as the patient's overall health. Common treatments include watchful waiting, radiation therapy, chemotherapy, immunotherapy, targeted therapy, or a combination of these approaches.

Epstein-Barr virus (EBV) infections, also known as infectious mononucleosis or "mono," is a viral infection that most commonly affects adolescents and young adults. The virus is transmitted through saliva and other bodily fluids, and can cause a variety of symptoms including fever, sore throat, swollen lymph nodes, fatigue, and skin rash.

EBV is a member of the herpesvirus family and establishes lifelong latency in infected individuals. After the initial infection, the virus remains dormant in the body and can reactivate later in life, causing symptoms such as fatigue and swollen lymph nodes. In some cases, EBV infection has been associated with the development of certain types of cancer, such as Burkitt's lymphoma and nasopharyngeal carcinoma.

The diagnosis of EBV infections is typically made based on a combination of clinical symptoms and laboratory tests, such as blood tests that detect the presence of EBV antibodies or viral DNA. Treatment is generally supportive and aimed at alleviating symptoms, as there is no specific antiviral therapy for EBV infections.

Asparaginase is a medication that is used in the treatment of certain types of cancer, such as acute lymphoblastic leukemia (ALL) and non-Hodgkin lymphoma (NHL). It is an enzyme that breaks down the amino acid asparagine, which is a building block of proteins. Some cancer cells are unable to produce their own asparagine and rely on obtaining it from the bloodstream. By reducing the amount of asparagine in the blood, asparaginase can help to slow or stop the growth of these cancer cells.

Asparaginase is usually given as an injection into a muscle (intramuscularly) or into a vein (intravenously). It may be given alone or in combination with other chemotherapy drugs. The specific dosage and duration of treatment will depend on the individual's medical history, the type and stage of cancer being treated, and how well the person tolerates the medication.

Like all medications, asparaginase can cause side effects. Common side effects include nausea, vomiting, loss of appetite, and changes in liver function tests. Less common but more serious side effects may include allergic reactions, pancreatitis, and blood clotting problems. It is important for patients to discuss the potential risks and benefits of asparaginase with their healthcare provider before starting treatment.

CD1 antigens are a group of molecules found on the surface of certain immune cells, including dendritic cells and B cells. They play a role in the immune system by presenting lipid antigens to T cells, which helps initiate an immune response against foreign substances such as bacteria and viruses. CD1 molecules are distinct from other antigen-presenting molecules like HLA because they present lipids rather than peptides. There are five different types of CD1 molecules (CD1a, CD1b, CD1c, CD1d, and CD1e) that differ in their tissue distribution and the types of lipid antigens they present.

Medical Definition of "Herpesvirus 4, Human" (Epstein-Barr Virus)

"Herpesvirus 4, Human," also known as Epstein-Barr virus (EBV), is a member of the Herpesviridae family and is one of the most common human viruses. It is primarily transmitted through saliva and is often referred to as the "kissing disease."

EBV is the causative agent of infectious mononucleosis (IM), also known as glandular fever, which is characterized by symptoms such as fatigue, sore throat, fever, and swollen lymph nodes. The virus can also cause other diseases, including certain types of cancer, such as Burkitt's lymphoma, Hodgkin's lymphoma, and nasopharyngeal carcinoma.

Once a person becomes infected with EBV, the virus remains in the body for the rest of their life, residing in certain white blood cells called B lymphocytes. In most people, the virus remains dormant and does not cause any further symptoms. However, in some individuals, the virus may reactivate, leading to recurrent or persistent symptoms.

EBV infection is diagnosed through various tests, including blood tests that detect antibodies against the virus or direct detection of the virus itself through polymerase chain reaction (PCR) assays. There is no cure for EBV infection, and treatment is generally supportive, focusing on relieving symptoms and managing complications. Prevention measures include practicing good hygiene, avoiding close contact with infected individuals, and not sharing personal items such as toothbrushes or drinking glasses.

Galactosylceramides are a type of glycosphingolipids, which are lipid molecules that contain a sugar (glyco-) attached to a ceramide. Galactosylceramides have a galactose molecule attached to the ceramide. They are important components of cell membranes and play a role in cell recognition and signaling. In particular, they are abundant in the myelin sheath, which is the protective covering around nerve fibers in the brain and spinal cord. Abnormal accumulation of galactosylceramides can lead to certain genetic disorders, such as Krabbe disease and Gaucher disease.

Follicular lymphoma is a specific type of low-grade or indolent non-Hodgkin lymphoma (NHL). It develops from the B-lymphocytes, a type of white blood cell found in the lymphatic system. This lymphoma is characterized by the presence of abnormal follicles or nodules in the lymph nodes and other organs. The neoplastic cells in this subtype exhibit a distinct growth pattern that resembles normal follicular centers, hence the name "follicular lymphoma."

The majority of cases involve a translocation between chromosomes 14 and 18 [t(14;18)], leading to an overexpression of the BCL-2 gene. This genetic alteration contributes to the cancer cells' resistance to programmed cell death, allowing them to accumulate in the body.

Follicular lymphoma is typically slow-growing and may not cause symptoms for a long time. Common manifestations include painless swelling of lymph nodes, fatigue, weight loss, and night sweats. Treatment options depend on various factors such as the stage of the disease, patient's age, and overall health. Watchful waiting, chemotherapy, immunotherapy, targeted therapy, radiation therapy, or a combination of these approaches may be used to manage follicular lymphoma.

'NK Cell Lectin-Like Receptor Subfamily B' refers to a group of genes that encode proteins found on natural killer (NK) cells, which are a type of white blood cell in the human body. These proteins belong to a larger family called C-type lectin receptors (CLRs), which are involved in various immune functions such as pathogen recognition and immune cell activation.

The NK Cell Lectin-Like Receptor Subfamily B includes several genes, such as NKp80, NKp46, and NKp30, that encode proteins expressed on the surface of NK cells. These proteins function as activating receptors, meaning they can trigger NK cell activation and subsequent immune responses when they bind to specific ligands on the surface of infected or abnormal cells.

Overall, the NK Cell Lectin-Like Receptor Subfamily B plays an essential role in the innate immune response against viral infections and cancer by mediating NK cell cytotoxicity and cytokine production.

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.

Burkitt lymphoma is a type of aggressive non-Hodgkin lymphoma (NHL), which is a cancer that originates in the lymphatic system. It is named after Denis Parsons Burkitt, an Irish surgeon who first described this form of cancer in African children in the 1950s.

Burkitt lymphoma is characterized by the rapid growth and spread of abnormal B-lymphocytes (a type of white blood cell), which can affect various organs and tissues, including the lymph nodes, spleen, liver, gastrointestinal tract, and central nervous system.

There are three main types of Burkitt lymphoma: endemic, sporadic, and immunodeficiency-associated. The endemic form is most common in equatorial Africa and is strongly associated with Epstein-Barr virus (EBV) infection. The sporadic form occurs worldwide but is rare, accounting for less than 1% of all NHL cases in the United States. Immunodeficiency-associated Burkitt lymphoma is seen in individuals with weakened immune systems due to HIV/AIDS or immunosuppressive therapy after organ transplantation.

Burkitt lymphoma typically presents as a rapidly growing mass, often involving the jaw, facial bones, or abdominal organs. Symptoms may include swollen lymph nodes, fever, night sweats, weight loss, and fatigue. Diagnosis is made through a biopsy of the affected tissue, followed by immunohistochemical staining and genetic analysis to confirm the presence of characteristic chromosomal translocations involving the MYC oncogene.

Treatment for Burkitt lymphoma typically involves intensive chemotherapy regimens, often combined with targeted therapy or immunotherapy. The prognosis is generally good when treated aggressively and promptly, with a high cure rate in children and young adults. However, the prognosis may be poorer in older patients or those with advanced-stage disease at diagnosis.

T-cell receptor (TCR) alpha genes are part of the human genome that contain the genetic information necessary for the development and function of alpha chains of the T-cell receptor. These receptors are found on the surface of T-cells, a type of white blood cell that plays a central role in the adaptive immune response. The TCR recognizes and binds to specific antigens presented in the context of major histocompatibility complex (MHC) molecules on the surface of infected or damaged cells, triggering an immune response.

The TCR alpha genes are located on chromosome 14 and consist of several variable (V), diversity (D), joining (J), and constant (C) gene segments. During the development of T-cells in the thymus, a process called V(D)J recombination randomly assembles these gene segments to generate a diverse repertoire of TCR alpha chains with unique antigen specificities. This allows the immune system to recognize and respond to a wide variety of potential threats.

Prognosis is a medical term that refers to the prediction of the likely outcome or course of a disease, including the chances of recovery or recurrence, based on the patient's symptoms, medical history, physical examination, and diagnostic tests. It is an important aspect of clinical decision-making and patient communication, as it helps doctors and patients make informed decisions about treatment options, set realistic expectations, and plan for future care.

Prognosis can be expressed in various ways, such as percentages, categories (e.g., good, fair, poor), or survival rates, depending on the nature of the disease and the available evidence. However, it is important to note that prognosis is not an exact science and may vary depending on individual factors, such as age, overall health status, and response to treatment. Therefore, it should be used as a guide rather than a definitive forecast.

CD1d is a type of antigen presenting molecule that is expressed on the surface of certain immune cells, including dendritic cells and B cells. Unlike classical MHC molecules, which present peptide antigens to T cells, CD1d presents lipid antigens to a specific subset of T cells called natural killer T (NKT) cells.

CD1d is composed of an alpha-helical heavy chain and a beta-2 microglobulin light chain, and it has a hydrophobic binding groove that can accommodate lipid antigens. CD1d-restricted NKT cells recognize and respond to these lipid antigens through their invariant T cell receptor (TCR), leading to the rapid production of cytokines and the activation of various immune responses.

CD1d-restricted NKT cells have been implicated in a variety of immunological functions, including the regulation of autoimmunity, antitumor immunity, and infectious disease.

Immunohistochemistry (IHC) is a technique used in pathology and laboratory medicine to identify specific proteins or antigens in tissue sections. It combines the principles of immunology and histology to detect the presence and location of these target molecules within cells and tissues. This technique utilizes antibodies that are specific to the protein or antigen of interest, which are then tagged with a detection system such as a chromogen or fluorophore. The stained tissue sections can be examined under a microscope, allowing for the visualization and analysis of the distribution and expression patterns of the target molecule in the context of the tissue architecture. Immunohistochemistry is widely used in diagnostic pathology to help identify various diseases, including cancer, infectious diseases, and immune-mediated disorders.

... primary effusion lymphoma; plasmablastic lymphoma; extranodal NK/T cell lymphoma, nasal type; peripheral T cell lymphoma, not ... T-cell large granular lymphocyte leukemia Aggressive NK cell leukemia Adult T-cell leukemia/lymphoma Extranodal NK/T-cell ... lymphoma, nasal type Enteropathy-associated T-cell lymphoma Hepatosplenic T-cell lymphoma Blastic NK cell lymphoma Mycosis ... "popcorn cells". (H&E) Certain lymphomas (extranodal NK/T-cell lymphoma, nasal type and type II enteropathy-associated T-cell ...
Hu B, Oki Y (2018). "Novel Immunotherapy Options for Extranodal NK/T-Cell Lymphoma". Frontiers in Oncology. 8: 139. doi:10.3389 ... of CD30-positive B cell lymphomas Various types of CD30-positive T cell lymphomas CD30-positive cases of the NK cell lymphoma, ... extranodal NK/T-cell lymphoma, nasal type CD30 has been shown to interact with TRAF5, and TRAF2. GRCh38: Ensembl release 89: ... Lee SY, Park CG, Choi Y (Feb 1996). "T cell receptor-dependent cell death of T cell hybridomas mediated by the CD30 cytoplasmic ...
Extranodal NK/T cell lymphoma, nasal type for comparison. These lymphoma cells are typically monotonous, with folded nuclei, ... Takahara M, Kumai T, Kishibe K, Nagato T, Harabuchi Y (2021). "Extranodal NK/T-Cell Lymphoma, Nasal Type: Genetic, Biologic, ... "Extranodal NK / T cell lymphoma, nasal type". Pathology Outlines.{{cite web}}: CS1 maint: multiple names: authors list (link) ... connective tissue with some seromucous glands and cells representing inflammation (mostly neutrophils and eosinophils). Polyps ...
... extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT). ENKTCL-NT is a rare type of lymphoma that commonly involves the nasal ... usually NK cells or, rarely, cytotoxic T cells. The disease is therefore now regarded as a NK/T cell malignancy, is grouped ... Hu B, Oki Y (2018). "Novel Immunotherapy Options for Extranodal NK/T-Cell Lymphoma". Frontiers in Oncology. 8: 139. doi:10.3389 ... Yamaguchi M, Oguchi M, Suzuki R (September 2018). "Extranodal NK/T-cell lymphoma: Updates in biology and management strategies ...
"UBE1 expression in extranodal NK/T cell lymphoma, nasal type". Leukemia & Lymphoma. 49 (9): 1821-2. doi:10.1080/ ... Cell. 125 (4): 801-14. doi:10.1016/j.cell.2006.03.032. PMID 16713569. Gallegos JR, Litersky J, Lee H, Sun Y, Nakayama K, ... Cell. 134 (2): 268-78. doi:10.1016/j.cell.2008.05.046. PMID 18662542. Lake MW, Wuebbens MM, Rajagopalan KV, Schindelin H ( ... Cell. 131 (2): 309-23. doi:10.1016/j.cell.2007.07.044. hdl:20.500.12110/paper_00928674_v131_n2_p309_CarbiaNagashima. PMID ...
Yamaguchi M, Oguchi M, Suzuki R (September 2018). "Extranodal NK/T-cell lymphoma: Updates in biology and management strategies ... Chan JY, Lim ST (April 2018). "Novel findings from the Asian Lymphoma Study Group: focus on T and NK-cell lymphomas". ... Enteropathy-associated T-cell lymphoma (EATL), previously termed enteropathy-associated T-cell lymphoma, type I and at one time ... and 16 of 19 patients with other types of T-cell/NK-cell lymphomas. A phase 1 study sponsored by the National Institutes of ...
... extranodal NK/T-cell lymphoma, nasal type. In 2010, a similarly non-malignant disorder that involved the proliferation of NK ... peripheral T-cell lymphoma not otherwise specified, T cell lymphomas that consist of mature T cells and occur in the GI tact, ... In contrast to NKCE and LG, extranodal NK/T cell lymphoma, nasal type: is malignant and often aggressive; causes serious, ... The two NK cell proliferation diseases have been termed NK cell enteropathy and lymphomatoid gastropathy with NKCE being seen ...
Extranodal NK/T-cell lymphoma, nasal type, Cutaneous T-cell lymphoma (CTCL), etc. T-cell lymphoma which develops from the lymph ... angioimmunoblastic T-cell lymphoma (AITL), extranodal NK/T-cell lymphoma, nasal type, and Peripheral T-cell lymphoma not ... Extranodal NK/T-cell lymphoma, nasal type (ENKTL): Aggressive T-cell lymphoma, usually associated with Epstein-Barr virus (EBV ... Angioimmunoblastic T-cell lymphoma (AITL): Aggressive form of T-cell lymphoma.[citation needed] Anaplastic large cell lymphoma ...
"Gene expression profiling identifies emerging oncogenic pathways operating in extranodal NK/T-cell lymphoma, nasal type". Blood ... Cell. 21 (5): 959-65. doi:10.1016/j.devcel.2011.08.015. PMID 22036506. Castillo-Lluva S, Tan CT, Daugaard M, Sorensen PH, ... Malliri A (March 2013). "The tumour suppressor HACE1 controls cell migration by regulating Rac1 degradation". Oncogene. 32 (13 ...
The immunophenotype of this disease is the same as extranodal NK/T-cell lymphoma, nasal type and is shown in the table below. ... The cell of origin is believed to be an NK cell. Blastoid NK cell lymphoma appears to be a different entity and shows no ... Aggressive NK-cell leukemia is a disease with an aggressive, systemic proliferation of natural killer cells (NK cells) and a ... Currently Aggressive NK-cell leukemia, being a subtype of PTCL, is treated similarly to B-cell lymphomas. However, in recent ...
... chronic active EBV infection or de novo extranodal natural killer (NK)/T-cell lymphoma, nasal type?" (PDF). Internal Medicine. ... Interstitial pneumonia Lymphoma, including B-cell, T-cell and NK-cell lymphomas Haemophagocytic syndrome Coronary artery ... most often the T-cells and NK cells in Asians/South Americans and the B-cells in the other racial groups. Various cytokine ... "Differences between T cell-type and natural killer cell-type chronic active Epstein-Barr virus infection". The Journal of ...
... adult T-cell leukaemia/lymphoma, enteropathy-type T-cell lymphoma, nasal NK/T-cell lymphoma, hepatosplenic gamma-delta T-cell ... Extranodal symptoms are symptoms shown by organs or tissues outside of the lymphatic system. Examples of such signs and ... Anaplastic large cell lymphoma (ALCL) is a subtype of mature T-cell lymphoma involving T-cells or natural killer (NK) cells, ... The common subtypes are angioimmunoblastic T-cell lymphoma, anaplastic large cell lymphoma and peripheral T-cell lymphoma not ...
... extranodal NK/T cell lymphoma, nasal type, rhabdomyosarcoma, Ewing/PNET, mucosal malignant melanoma and neuroendocrine ... The olfactory epithelium consists of olfactory sensory cells, sustentacular cells and basal cells. Esthesioneuroblastoma ... Arising from the upper nasal tract, esthesioneuroblastoma is believed to originate from sensory neuroepithelial cells, also ... Esthesioneuroblastoma is of neurocrest origin, arising from olfactory sensory cells in the olfactory epithelium. ...
... aggressive NK-cell leukemia (also termed aggressive NK-cell leukemia/lymphoma), and extranodal NK/T-cell lymphoma, nasal type. ... NK/T cell lymphoma, or aggressive NK cell lymphoma generally require chemotherapeutic regimens directed at these complications ... The bite sites are infiltrated with T helper cells, CD8+ T cells, and CD16+ NK cells. In this mixture of infiltrates, most of ... In rare cases, mantle cell lymphoma, a subtype of B-cell lymphomas, has been reported in association with mosquito bite ...
Extranodal NK/T cell lymphoma, nasal type (ENKTCL-NT): ENKTCL-NT is a lymphoma that usually involves nasal and oropharyngeal ... Tang XF, Yang L, Duan S, Guo H, Guo QN (December 2018). "Intestinal T-cell and NK/T-cell lymphomas: A clinicopathological study ... Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS): PTCL-NOS is a heterogenous group of T cell lymphomas that ... Anaplastic large cell lymphoma, ALK positive (ALCL,ALK+): ALCL,ALK+ is a subtype of anaplastic large cell lymphoma. It commonly ...
... extranodal marginal zone B cell lymphoma, extranodal NK/T-cell lymphoma, nasal type, peripheral T-cell lymphoma, activin ... Primary testicular diffuse large B-cell lymphoma (PT-DLBCL), also termed testicular diffuse large B-cell lymphoma and diffuse ... Ollila TA, Olszewski AJ (June 2018). "Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of ... large B-cell lymphoma of the testes, is a variant of the diffuse large B-cell lymphomas (DLBCL). DLBCL are a large and diverse ...
... extranodal NK-T-cell lymphoma and diffuse large B-cell lymphoma. Human T-cell leukemia virus: associated with adult T-cell ... Hepatitis C virus: associated with splenic marginal zone lymphoma, lymphoplasmacytic lymphoma and diffuse large B-cell lymphoma ... associated with anaplastic large cell lymphoma. Familial lymphoid cancer is rare. The familial risk of lymphoma is elevated for ... Lymphomas are types of cancer that develop from lymphocytes, a type of white blood cell. Risk factors include poor immune ...
... extranodal NK/T-cell lymphoma, nasal type, pancreatic acinar cell carcinoma, pheochromocytoma, paraganglioma, small cell lung ... also present on subset of CD4+ T cells and CD8+ T cells. In cell adhesion, CD56 contributes to cell-cell adhesion or cell- ... Τ cells and activated CD8+ T cells, as well as on dendritic cells. NCAM has been implicated as having a role in cell-cell ... Normal cells that stain positively for CD56 include NK cells, activated T cells, the brain and cerebellum, and neuroendocrine ...
NK) cell neoplasms: aggressive NK cell leukemia and extranodal NK cell lymphoma, nasal type". Ann. Oncol. 21 (5): 1032-40. doi: ... In some cases, NK cell therapy is a choice. NK cells are known for their ability to eradicate tumor cells without any prior ... To this end, lymphoid leukemias can also be divided by the type of cells affected: B-cell leukemia T-cell leukemia NK-cell ... "Nasal-type NK/T-cell lymphomas are more frequently T rather than NK lineage based on T-cell receptor gene, RNA, and protein ...
Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT]: Unlike ITCLD-GT, ENKTCL-NT a) is an Epstein-Barr virus-associated ... Peripheral T-cell lymphoma subtype peripheral T-cell lymphoma not otherwise specified (PTCL-NOS): Unlike ITCLD-GT, PTCL-NOS a) ... Tse E, Kwong YL (April 2017). "The diagnosis and management of NK/T-cell lymphomas". Journal of Hematology & Oncology. 10 (1): ... Unlike certain types of T cell lymphomas for which it has been mistaken, the T cells in ITCLD-GT do not express the neutral ...
... lymphoproliferative disorder Severe mosquito bite allergy Adult T-cell leukaemia/lymphoma Extranodal NK/T-cell lymphoma, nasal ... large B-cell lymphoma Intravascular large B-cell lymphoma ALK-positive large B-cell lymphoma Plasmablastic lymphoma Primary ... NOS Angioimmunoblastic T-cell lymphoma Follicular T-cell lymphoma Nodal peripheral T-cell lymphoma with T follicular helper ... leukaemia Chronic lymphoproliferative disorder of NK cells Aggressive NK-cell leukaemia Systemic EBV-positive T-cell lymphoma ...
... lymphomas variants Intravascular large B-cell lymphoma Intravascular NK-cell lymphoma Intravascular T-cell lymphoma Primary ... Determining the extent of the disease (localized or advanced; nodal or extranodal) The person's general health status. ... large B cell lymphoma T cell/histiocyte-rich large B-cell lymphoma Primary cutaneous diffuse large B-cell lymphoma, leg type ( ... The B-cell lymphomas are types of lymphoma affecting B cells. Lymphomas are "blood cancers" in the lymph nodes. They develop ...
Dojcinov SD, Fend F, Quintanilla-Martinez L (March 2018). "EBV-Positive Lymphoproliferations of B- T- and NK-Cell Derivation in ... 369 The disease usually has an extranodal presentation, with or without lymph node involvement.: 369 Morphologically, areas of ... one with monotonous large cells, the other with numerous cell sizes as well as reactive cells, but different clinical behavior ... Adam P, Bonzheim I, Fend F, Quintanilla-Martínez L (2011). "Epstein-Barr virus-positive diffuse large B-cell lymphomas of the ...
Adult T-cell leukemia/lymphoma Angiocentric lymphoma (extranodal natural killer cell lymphoma, nasal-type NK lymphoma, NK/T- ... Blastic NK-cell lymphoma CD30+ cutaneous T-cell lymphoma (primary cutaneous anaplastic large cell lymphoma) Cutaneous lymphoid ... Diffuse large B-cell lymphoma (primary cutaneous large B-cell lymphoma) Granulocytic sarcoma (chloroma, myeloid sarcoma) ... Subcutaneous T-cell lymphoma (panniculitis-like T-cell lymphoma) Vesiculopustular eruption and leukemoid reaction in Down ...
Peripheral T cell lymphomas (PTCL) are a group of NK-cell or T-cell malignancies that include extranodal NK/T cell lymphoma, ... cell leukemia (also termed aggressive NK-cell leukemia/lymphoma), and extranodal NK/T-cell lymphoma, nasal type, as well as in ... "Extranodal NK/T cell lymphoma and aggressive NK cell leukaemia: evidence for their origin on CD56+bright CD16-/+dim NK cells". ... A very high density of EBV+ NK cells in these lesions suggests the disorder has progressed to NK/T cell lymphoma or NK cell ...
The main forms of T cell lymphoma are: Extranodal T cell lymphoma Cutaneous T cell lymphomas: Sézary syndrome and Mycosis ... These cytokines influence the effector functions of other cells, in particular macrophages and NK cells. Antigen-naive T cells ... Cytotoxic T cells (TC cells, CTLs, T-killer cells, killer T cells) destroy virus-infected cells and tumor cells, and are also ... T helper cells (TH cells) assist other lymphocytes, including the maturation of B cells into plasma cells and memory B cells, ...
... nasal and nasal type Extranodal NK/T cell lymphoma, nasal type T/NK-cell lymphoma M9727/3 Precursor cell lymphoblastic lymphoma ... cell lymphoma Hepatosplenic T-cell lymphoma M9717/3 Intestinal T-cell lymphoma Enteropathy type T-cell lymphoma M9718/3 Primary ... Angiotropic lymphoma T-cell rich large B-cell lymphoma Histiocyte-rich large B-cell lymphoma Anaplastic large B-cell lymphoma ... NOS Peripheral T-cell lymphoma, NOS T-cell lymphoma, NOS Peripheral T-cell lymphoma, pleomorphic small/medium/large cell/T-zone ...
Ollila TA, Olszewski AJ (June 2018). "Extranodal Diffuse Large B Cell Lymphoma: Molecular Features, Prognosis, and Risk of ... Dojcinov SD, Fend F, Quintanilla-Martinez L (March 2018). "EBV-Positive Lymphoproliferations of B- T- and NK-Cell Derivation in ... Germinal center B-cell like diffuse large B-cell lymphoma "A clinical evaluation of the International Lymphoma Study Group ... Bone marrow involvement may be due to DLBCL, NOS cells or low grade lymphoma cells; only DLBCL, NOS cell infiltrates indicate a ...
"Orbital signet-ring cell lymphoma of diffuse, large, B-cell type". Can J Ophthalmol. 48 (6): e156-8. doi:10.1016/j.jcjo.2013.08 ... extra-nodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT) - Type". Saudi J Ophthalmol. 28 (4): 338-40. doi ... S2CID 10024047.{{cite journal}}: CS1 maint: multiple names: authors list (link) Das D, Kaur I, Ali MJ, Biswas NK, Das S, Kumar ... "Ocular adnexal diffuse large B-cell lymphoma: a multicenter international study". JAMA Ophthalmol. 133 (2): 165-73. doi:10.1001 ...

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