Polycythemia Vera
Thrombocythemia, Essential
Janus Kinase 2
Primary Myelofibrosis
Myeloproliferative Disorders
Erythropoietin
Erythroid Precursor Cells
Phlebotomy
Erythropoiesis
Receptors, Erythropoietin
Hematocrit
Receptors, Thrombopoietin
Bloodletting
Von Hippel-Lindau Tumor Suppressor Protein
Hematopoiesis, Extramedullary
Granulocytes
Erythrocyte Volume
Somatostatinoma
GPI-Linked Proteins
Hydroxyurea
Mutation, Missense
Mutation
Amino Acid Substitution
Isoantigens
Cyanosis
Erythroid Cells
Colony-Forming Units Assay
Paraganglioma
Erythrocyte Indices
Bone Marrow
Heterozygote
Budd-Chiari Syndrome
Hemoglobins
Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative
Chromosomes, Human, 19-20
Insulin-like growth factor I plays a role in regulating erythropoiesis in patients with end-stage renal disease and erythrocytosis. (1/419)
Erythroid progenitor growth, the serum hormones that regulate erythropoiesis, and the effect of patient's serum on the growth of normal erythroid progenitors were assessed in eight patients with end-stage renal disease (ESRD) and erythrocytosis. All patients were male and had been on maintenance dialysis, they had a hematocrit >50% and/or a red blood cell count >6 x 10(12)/L and an arterial oxygen saturation >95%. Four had acquired cystic disease of the kidney (ACDK), and four other non-ACDK patients did not have known causes of secondary erythrocytosis after appropriate investigations and long-term follow-up. The methylcellulose culture technique was used to assay the erythroid progenitor (BFU-E/CFU-E) growth. Serum erythropoietin (EPO) and insulin-like growth factor I (IGF-I) levels were measured by RIA. Paired experiments were performed to determine the effects of 10% sera from ESRD patients and control subjects on normal marrow CFU-E growth. The numbers of EPO-dependent BFU-E in marrow and/or blood of patients with ESRD and erythrocytosis were higher than those of normal controls. No EPO-independent erythroid colonies were found. Serum EPO levels were constantly normal in one patient and elevated in three patients with ACDK; for non-ACDK patients, EPO levels were normal or low in two patients and persistently increased in one, but fluctuated in the remaining one on serial assays. There was no correlation between serum EPO levels and hematocrit values. The serum IGF-I levels in patients with ESRD and erythrocytosis were significantly increased compared with normal subjects or ESRD patients with anemia. We found an inverse correlation between serum EPO and IGF-I levels. Sera from patients with ESRD and erythrocytosis exhibited a stimulating effect on normal marrow CFU-E growth. The stimulating effect of sera from patients who had a normal serum EPO level and an elevated IGF-I level could be partially blocked by anti-IGF-I. The present study suggests that IGF-I plays an important role in the regulation of erythropoiesis in patients with ESRD and erythrocytosis who did not have an increased EPO production. (+info)Pulmonary prostacyclin synthase overexpression in transgenic mice protects against development of hypoxic pulmonary hypertension. (2/419)
Prostacyclin synthase (PGIS) is the final committed enzyme in the metabolic pathway leading to prostacyclin (PGI2) production. Patients with severe pulmonary hypertension have a PGIS deficiency of their precapillary vessels, but the importance of this deficiency for lung vascular remodeling remains unclear. We hypothesized that selective pulmonary overexpression of PGIS may prevent the development of pulmonary hypertension. To study this hypothesis, transgenic mice were created with selective pulmonary PGIS overexpression using a construct of the 3.7-kb human surfactant protein-C (SP-C) promoter and the rat PGIS cDNA. Transgenic mice (Tg+) and nontransgenic littermates (Tg-) were subjected to a simulated altitude of 17,000 ft for 5 weeks, and right ventricular systolic pressure (RVSP) was measured. Histology was performed on the lungs. The Tg+ mice produced 2-fold more pulmonary 6-keto prostaglandin F1alpha (PGF1alpha) levels than did Tg- mice. After exposure to chronic hypobaric hypoxia, Tg+ mice have lower RVSP than do Tg- mice. Histologic examination of the lungs revealed nearly normal arteriolar vessels in the Tg+ mice in comparison with vessel wall hypertrophy in the Tg- mice. These studies demonstrate that Tg+ mice were protected from the development of pulmonary hypertension after exposure to chronic hypobaric hypoxia. We conclude that PGIS plays a major role in modifying the pulmonary vascular response to chronic hypoxia. This has important implications for the pathogenesis and treatment of severe pulmonary hypertension. (+info)Localization of the gene responsible for familial benign polycythemia to chromosome 11q23. (3/419)
Familial benign polycythemia (FBP) (OMIM 263400) is a rare autosomal recessive condition characterized by erythrocytosis, normal leukocyte and platelet counts, normal uric acid level, and usually increased erythropoietin production. There is a high incidence of this disorder in Chuvashia (Russian Federation), probably due to a founder effect. In an attempt to locate the gene responsible for this disorder, we have carried out linkage studies in 12 Chuvash families, with 35 affected and 32 unaffected members. Linkage to the erythropoietin and erythropoietin receptor loci was excluded, and the FBP gene was assigned to the region of chromosome 11q23 between D11S4142 and D11S1356, with a maximal lod score of 6.61. (+info)Activation of the erythropoietin receptor by the gp55-P viral envelope protein is determined by a single amino acid in its transmembrane domain. (4/419)
The spleen focus forming virus (SFFV) gp55-P envelope glycoprotein specifically binds to and activates murine erythropoietin receptors (EpoRs) coexpressed in the same cell, triggering proliferation of erythroid progenitors and inducing erythroleukemia. Here we demonstrate specific interactions between the single transmembrane domains of the two proteins that are essential for receptor activation. The human EpoR is not activated by gp55-P but by mutation of a single amino acid, L238, in its transmembrane sequence to its murine counterpart serine, resulting in its ability to be activated. The converse mutation in the murine EpoR (S238L) abolishes activation by gp55-P. Computational searches of interactions between the membrane-spanning segments of murine EpoR and gp55-P provide a possible explanation: the face of the EpoR transmembrane domain containing S238 is predicted to interact specifically with gp55-P but not gp55-A, a variant which is much less effective in activating the murine EpoR. Mutational studies on gp55-P M390, which is predicted to interact with S238, provide additional support for this model. Mutation of M390 to isoleucine, the corresponding residue in gp55-A, abolishes activation, but the gp55-P M390L mutation is fully functional. gp55-P is thought to activate signaling by the EpoR by inducing receptor oligomerization through interactions involving specific transmembrane residues. (+info)Erythropoietin receptor mutations associated with familial erythrocytosis cause hypersensitivity to erythropoietin in the heterozygous state. (5/419)
Inherited mutations in the erythropoietin receptor (EPOR) causing premature termination of the receptor cytoplasmic region are associated with dominant familial erythrocytosis (FE), a benign clinical condition characterized by hypersensitivity of erythroid progenitor cells to EPO and low serum EPO (S-EPO) levels. We describe a Swedish family with dominant FE in which erythrocytosis segregates with a new truncation in the negative control domain of the EPOR. We show that cells engineered to concomitantly express the wild-type (WT) EPOR and mutant EPORs associated with FE (FE EPORs) are hypersensitive to EPO-stimulated proliferation and activation of Jak2 and Stat5. These results demonstrate that FE is caused by hyperresponsiveness of receptor-mediated signaling pathways and that this is dominant with respect to WT EPOR signaling. (+info)Role of the spleen in the exaggerated polycythemic response to hypoxia in chronic mountain sickness in rats. (6/419)
In a rat model of chronic mountain sickness, the excessive polycythemic response to hypoxic exposure is associated with profound splenic erythropoiesis. We studied the uptake and distribution of radioactive iron and red blood cell (RBC) morphology in intact and splenectomized rats over a 30-day hypoxic exposure. Retention of (59)Fe in the plasma was correlated with (59)Fe uptake by both spleen and marrow and the appearance of (59)Fe-labeled RBCs in the blood. (59)Fe uptake in both the spleen and the marrow paralleled the production of nucleated RBCs. Splenic (59)Fe uptake was approximately 10% of the total marrow uptake under normoxic conditions but increased to 60% of the total marrow uptake during hypoxic exposure. Peak splenic (59)Fe uptake and splenomegaly occurred at the most intense phase of erythropoiesis and coincided with the rapid appearance of (59)Fe-labeled RBCs in the blood. The bone marrow remains the most important erythropoietic organ under both resting and stimulated states, but inordinate splenic erythropoiesis in this rat strain accounts in large measure for the excessive polycythemia during the development of chronic mountain sickness in chronic hypoxia. (+info)Neutrophil alkaline phosphatase score in chronic granulocytic leukaemia: effects of splenectomy and antileukaemic drugs. (7/419)
Staining with naphthol AS phosphate and Fast Blue BB salt has been used for the estimation of neutrophil alkaline phosphatase (NAP) scores in patients with chronic granulocytic leukaemia (CGL). The very low scores found at diagnosis rise when the disease is treated, and there is some inverse correlation between the NAP score and the absolute neutrophil count. Patients treated intensively developed high NAP scores. Elective splenectomy performed during the chronic phase of CGL is followed by a pronounced but transient neutrophilia and a concurrent striking rise in the NAP score. Similar changes were observed in patients without CGL who underwent splenectomy. These observations can be explained by assuming that newly formed neutrophils in CGL have a normal content of NAP but are rapidly sequestered in non-circulating extramedullary pools, whereas the circulating neutrophil with a typically low NAP content is a relatively aged cell which has lost enzyme activity. In subjects with or without CGL, removal of the spleen, a major site of such pooling, temporarily permits the circulation of newly formed neutrophils but eventually other organs assume the sequestering functions of the spleen. Thus the aberrations of NAP score seen in CGL might be attributable not to an intrinsic cellular defect but to an exaggeration of the granulocyte storage phenomena which also occur in subjects without CGL. (+info)Significance of an abnormally low or high hemoglobin concentration during pregnancy: special consideration of iron nutrition. (8/419)
An association between moderate anemia and poor perinatal outcomes has been found through epidemiologic studies, although available evidence cannot establish this relation as causal. Anemia may not be a direct cause of poor pregnancy outcomes, except in the case of maternal mortality resulting directly from severe anemia due to hypoxia and heart failure. Preventing or treating anemia, whether moderate or severe, is desirable. Because iron deficiency is a common cause of maternal anemia, iron supplementation is a common practice to reduce the incidence of maternal anemia. Nevertheless, the effectiveness of large-scale supplementation programs needs to be improved operationally and, where multiple micronutrient deficiencies are common, supplementation beyond iron and folate can be considered. High hemoglobin concentrations are often mistaken as adequate iron status; however, high hemoglobin is independent of iron status and is often associated with poor health outcomes. Very high hemoglobin concentrations cause high blood viscosity, which results in both compromised oxygen delivery to tissues and cerebrovascular complications. Epidemiologic studies have also found an association between high maternal hemoglobin concentrations and an increased risk of poor pregnancy outcomes. Evidence does not suggest that this association is causal; it could be better attributed to hypertensive disorders of pregnancy and to preeclampsia. The pathophysiologic mechanism of these conditions during pregnancy can produce higher hemoglobin concentrations because of reduced normal plasma expansion and cause fetal stress because of reduced placental-fetal perfusion. Accordingly, higher than normal hemoglobin concentrations should be regarded as an indicator of possible pregnancy complications, not necessarily as a sign of adequate iron nutrition, because iron supplementation does not increase hemoglobin higher than the optimal concentration needed for oxygen delivery. (+info)The exact cause of polycythemia vera is not known, but it is believed to be due to a genetic mutation in the JAK2 gene, which is involved in the signaling pathways that regulate blood cell production. The condition typically affects adults over the age of 60 and is more common in men than women.
Symptoms of polycythemia vera can include:
* Fatigue
* Weakness
* Shortness of breath
* Headaches
* Dizziness
* Itching
* Night sweats
* Weight loss
Diagnosis of polycythemia vera is typically made based on a combination of physical examination, medical history, and laboratory tests, including:
* Complete blood count (CBC) to measure the levels of red blood cells, white blood cells, and platelets
* Blood chemistry tests to assess liver function and other body chemicals
* Genetic testing to look for the JAK2 mutation
* Bone marrow biopsy to examine the bone marrow tissue for abnormalities
Treatment for polycythemia vera usually involves phlebotomy (the removal of blood from the body) to reduce the number of red blood cells and relieve symptoms such as itching and night sweats. In some cases, medications may be used to reduce the production of blood cells or to treat specific symptoms. Regular monitoring by a healthcare provider is important to detect any changes in the condition and to prevent complications.
Overall, polycythemia vera is a chronic and progressive disease that can have significant impact on quality of life if left untreated. Early diagnosis and appropriate treatment can help manage symptoms and improve outcomes for patients with this condition.
There are three main types of polycythemia:
1. Polycythemia vera (PV): This is the most common type and is characterized by an overproduction of red blood cells, white blood cells, and platelets. It is a slowly progressing disease that can lead to complications such as blood clots, bleeding, and an increased risk of cancer.
2. Essential thrombocythemia (ET): This type is characterized by an overproduction of platelets, which can increase the risk of blood clots and other cardiovascular problems.
3. Primary myelofibrosis (PMF): This type is characterized by bone marrow scarring, anemia, fatigue, and an increased risk of blood clots.
Symptoms of polycythemia may include:
* Headache
* Dizziness
* Fatigue
* Shortness of breath
* Pale skin
* Swelling in the spleen or liver
Diagnosis is based on a physical examination, medical history, and laboratory tests such as complete blood counts (CBCs) and bone marrow biopsies. Treatment options for polycythemia include:
1. Phlebotomy (removal of blood): This is the most common treatment for PV and ET, which involves removing excess blood to reduce the number of red blood cells, white blood cells, and platelets.
2. Chemotherapy: This may be used in combination with phlebotomy to treat PV and PMF.
3. Hydroxyurea: This medication is used to reduce the production of blood cells and relieve symptoms such as headache and dizziness.
4. Interferons: These medications are used to treat ET and may be effective in reducing the number of platelets.
5. Stem cell transplantation: In severe cases of PV or PMF, a stem cell transplant may be necessary.
It is important to note that these treatments do not cure polycythemia, but they can help manage symptoms and slow the progression of the disease. Regular monitoring and follow-up with a healthcare provider is essential to ensure the best possible outcomes.
1. Primary essential thrombocythemia (PET): This is the more common form, usually occurring spontaneously without any identifiable cause. Symptoms may include headache, migraine, seizures, and stroke-like episodes.
2. Secondary essential thrombocythemia: This form is caused by another medical condition or medication that stimulates the production of platelets. Symptoms are similar to those of PET, but there may be an underlying cause such as a tumor or an adverse reaction to medication.
Treatment for essential thrombocythemia includes medications to reduce platelet count and prevent blood clots, as well as close monitoring and management of any underlying causes. In some cases, surgery may be necessary to remove a tumor or other contributing factor.
PMF is a chronic disease that worsens over time, and it can lead to complications such as bleeding, infection, and bone damage. Treatment options include medications to reduce symptoms and slow the progression of the disease, as well as blood transfusions and splenectomy (removal of the spleen) in severe cases. The median age at diagnosis is around 60 years old, and the disease affects approximately 2-5 cases per million people per year.
Sources:
* American Cancer Society. (2019). What is primary myelofibrosis? Retrieved from
* Leukemia and Lymphoma Society. (n.d.). Primary Myelofibrosis. Retrieved from
There are several types of MPDs, including:
1. Polycythemia vera (PV): This is a rare disorder characterized by an overproduction of red blood cells, white blood cells, and platelets.
2. Essential thrombocythemia (ET): This is a rare disorder characterized by an overproduction of platelets.
3. Primary myelofibrosis (PMF): This is a rare and severe disorder characterized by the accumulation of scar tissue in the bone marrow, leading to an overproduction of immature white blood cells.
4. Chronic myelogenous leukemia (CML): This is a type of cancer that affects the bone marrow and blood cells, characterized by the overproduction of immature white blood cells.
The symptoms of MPDs can vary depending on the specific disorder, but may include:
* Fatigue
* Weakness
* Shortness of breath
* Headaches
* Dizziness
* Pale skin
* Easy bruising or bleeding
* Swollen spleen
* Bone pain
The exact cause of MPDs is not known, but they are thought to be due to genetic mutations that occur in the bone marrow cells. Treatment options for MPDs include:
* Chemotherapy: This is a type of drug that kills cancer cells.
* Radiation therapy: This is a type of treatment that uses high-energy X-rays to kill cancer cells.
* Stem cell transplantation: This is a procedure in which healthy stem cells are transplanted into the body to replace damaged or diseased bone marrow cells.
Overall, MPDs are rare and complex disorders that can have a significant impact on quality of life. While there is no cure for these conditions, treatment options are available to help manage symptoms and improve outcomes.
In healthy individuals, the normal platelet count ranges from 150,000 to 450,000 platelets per microliter of blood. In thrombocytosis, the platelet count is significantly higher than this range, often above 600,000 platelets/μL.
Thrombocytosis can be caused by a variety of factors, including:
1. Bone marrow disorders: Disorders such as essential thrombocythemia, polycythemia vera, and myelofibrosis can lead to an overproduction of platelets in the bone marrow.
2. Infection: Sepsis and other infections can cause a temporary increase in platelet production.
3. Inflammation: Certain inflammatory conditions, such as appendicitis and pancreatitis, can also lead to thrombocytosis.
4. Cancer: Some types of cancer, such as leukemia and lymphoma, can cause an overproduction of platelets.
5. Medications: Certain medications, such as estrogens and corticosteroids, can increase platelet production.
Thrombocytosis can lead to a range of complications, including:
1. Blood clots: The excessive number of platelets in the blood can increase the risk of blood clots forming in the veins and arteries.
2. Pulmonary embolism: If a blood clot forms in the lungs, it can cause a pulmonary embolism, which can be life-threatening.
3. Stroke: Thrombocytosis can increase the risk of stroke, especially if there are existing risk factors such as high blood pressure or a history of cardiovascular disease.
4. Heart attack and heart failure: Excessive platelet activity can increase the risk of heart attack and heart failure.
5. Gastrointestinal bleeding: The increased number of platelets in the blood can make it more difficult to control bleeding, especially in the gastrointestinal tract.
Thrombocytosis is typically diagnosed through a combination of physical examination, medical history, and laboratory tests, such as:
1. Complete blood count (CBC): This test measures the number of platelets in the blood, as well as other components such as red and white blood cells.
2. Blood smear: A sample of blood is examined under a microscope to assess the shape and size of the platelets.
3. Bone marrow aspiration and biopsy: These tests involve removing a small sample of bone marrow tissue to examine the number and type of cells present.
4. Imaging studies: Imaging tests such as ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) may be used to look for evidence of blood clots or other complications.
Treatment for thrombocytosis depends on the underlying cause and the severity of the condition. Some common treatments include:
1. Medications: Drugs such as aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), and blood thinners may be used to reduce the risk of blood clots and other complications.
2. Plateletpheresis: This is a procedure in which the patient's blood is removed and the platelets are separated from the rest of the blood components. The remaining blood is then returned to the body.
3. Splenectomy: In some cases, surgical removal of the spleen may be necessary to treat thrombocytosis.
4. Chemotherapy: This is a treatment that uses drugs to kill cancer cells, which can cause thrombocytosis in some cases.
Overall, it is important to seek medical attention if you experience any symptoms of thrombocytosis, as early diagnosis and treatment can help prevent complications and improve outcomes.
Somatostatinomas are relatively rare, accounting for only about 1-2% of all pancreatic tumors. They tend to affect older adults, typically in their 60s or 70s, and are more common in women than men. The exact cause of somatostatinomas is not well understood, but genetic mutations and changes in the DNA of somatostatin-producing cells may play a role in their development.
The diagnosis of a somatostatinoma typically involves a combination of imaging tests such as CT scans, MRI, and PET scans, along with blood tests to measure hormone levels and identify genetic mutations. Treatment options for somatostatinomas may include surgery, chemotherapy, and radiation therapy, depending on the size, location, and aggressiveness of the tumor. Prognosis for somatostatinoma patients is generally good if the tumor is diagnosed early and treated appropriately, but the long-term outlook can vary depending on the specific characteristics of the tumor and the individual patient's overall health.
There are several types of thrombosis, including:
1. Deep vein thrombosis (DVT): A clot forms in the deep veins of the legs, which can cause swelling, pain, and skin discoloration.
2. Pulmonary embolism (PE): A clot breaks loose from another location in the body and travels to the lungs, where it can cause shortness of breath, chest pain, and coughing up blood.
3. Cerebral thrombosis: A clot forms in the brain, which can cause stroke or mini-stroke symptoms such as weakness, numbness, or difficulty speaking.
4. Coronary thrombosis: A clot forms in the coronary arteries, which supply blood to the heart muscle, leading to a heart attack.
5. Renal thrombosis: A clot forms in the kidneys, which can cause kidney damage or failure.
The symptoms of thrombosis can vary depending on the location and size of the clot. Some common symptoms include:
1. Swelling or redness in the affected limb
2. Pain or tenderness in the affected area
3. Warmth or discoloration of the skin
4. Shortness of breath or chest pain if the clot has traveled to the lungs
5. Weakness, numbness, or difficulty speaking if the clot has formed in the brain
6. Rapid heart rate or irregular heartbeat
7. Feeling of anxiety or panic
Treatment for thrombosis usually involves medications to dissolve the clot and prevent new ones from forming. In some cases, surgery may be necessary to remove the clot or repair the damaged blood vessel. Prevention measures include maintaining a healthy weight, exercising regularly, avoiding long periods of immobility, and managing chronic conditions such as high blood pressure and diabetes.
In medicine, cyanosis is often used as an indication of the severity of a patient's condition. For example, a patient with severe cyanosis may have a more serious underlying condition than a patient with mild cyanosis. Additionally, cyanosis can be used to monitor the effectiveness of treatment and to determine when further interventions are necessary.
Cyanosis can be diagnosed through physical examination, blood tests, and other diagnostic procedures such as pulse oximetry or arterial blood gas analysis. Treatment for cyanosis depends on the underlying cause and may include oxygen therapy, medication, or surgical intervention.
In summary, cyanosis is a condition characterized by a bluish discoloration of the skin and mucous membranes due to inadequate oxygenation of the body's tissues. It is an important sign of underlying disease and can be used to assess the severity of a patient's condition and monitor the effectiveness of treatment.
Paragangliomas are rare, accounting for less than 1% of all tumors diagnosed in adults. They can occur at any age but are more common in young adults and middle-aged individuals. These tumors are more common in males than females, and their incidence is higher in certain families with inherited syndromes, such as neurofibromatosis type 1 (NF1) or familial paraganglioma.
The symptoms of paraganglioma depend on their location and size. Small tumors may not cause any symptoms, while larger tumors can press on nearby organs and structures, causing a variety of symptoms such as:
* Pain in the abdomen or pelvis
* Swelling or lump in the neck or abdomen
* High blood pressure
* Headaches
* Blurred vision
* Confusion or seizures (in cases of malignant paraganglioma)
Paragangliomas are difficult to diagnose, as they can be mistaken for other conditions such as appendicitis or pancreatitis. Imaging studies such as CT or MRI scans are often used to help identify the location and size of the tumor, while laboratory tests may be used to evaluate hormone levels and other factors that can help differentiate paraganglioma from other conditions.
Treatment for paraganglioma depends on the type, size, and location of the tumor, as well as the patient's overall health status. Small, benign tumors may not require treatment, while larger or malignant tumors may be treated with surgery, chemotherapy, or radiation therapy. In some cases, a combination of these treatments may be used.
The prognosis for paraganglioma is generally good if the tumor is diagnosed and treated early, but it can be poor if the tumor is large or has spread to other parts of the body. With surgical removal of the tumor, the 5-year survival rate is approximately 90% for patients with benign paraganglioma and 30-50% for those with malignant paraganglioma. However, the overall prognosis can vary depending on individual factors such as the size and location of the tumor, the effectiveness of treatment, and the patient's underlying health status.
White blood cells are an important part of the immune system and play a crucial role in fighting off infections and diseases. However, when there is an excessive increase in their numbers, it can lead to various complications, including:
1. Increased risk of infection: With too many white blood cells in the bloodstream, there is a higher chance of developing infections.
2. Inflammation: Excessive production of white blood cells can cause inflammation in various parts of the body.
3. Blood clotting disorders: White blood cells can clump together and form clots, which can lead to blockages in blood vessels.
4. Tissue damage: The excessive growth of white blood cells can cause damage to tissues and organs.
5. Bone marrow failure: Prolonged leukocytosis can lead to bone marrow failure, which can result in a decrease in the production of other blood cells, such as red blood cells and platelets.
There are several types of leukocytosis, including:
1. Reactive leukocytosis: This is the most common type and is caused by an infection or inflammation.
2. Chronic leukocytosis: This type is characterized by a persistent increase in white blood cells over a long period of time.
3. Acute leukocytosis: This type is characterized by a sudden and severe increase in white blood cells, often accompanied by other symptoms such as fever and fatigue.
4. Leukemia: This is a type of cancer that affects the bone marrow and blood cells. It can cause an abnormal increase in white blood cells.
Diagnosis of leukocytosis typically involves a physical examination, medical history, and laboratory tests such as complete blood count (CBC) and bone marrow biopsy. Treatment depends on the underlying cause and may include antibiotics for infections, steroids to reduce inflammation, or chemotherapy for leukemia. In some cases, no treatment is necessary if the condition resolves on its own.
The condition is named after the German physician Hans von Budde and the Italian physician Giorgio Chiari, who independently described it in the late 19th century. It is also known as Budd-Chiari syndrome or venous sinus thrombosis.
The exact cause of Budd-Chiari Syndrome is not known, but it is thought to be related to a combination of genetic and environmental factors. Some cases have been linked to autoimmune disorders, such as lupus, or to infections, such as endocarditis.
Symptoms of Budd-Chiari Syndrome can vary in severity and may include:
* Headaches
* Facial swelling
* Difficulty swallowing
* Numbness or tingling in the face or limbs
* Vision problems
* Fatigue
* Shortness of breath
If you suspect that you or someone else may have Budd-Chiari Syndrome, it is important to seek medical attention as soon as possible. A healthcare provider can perform a physical examination and order diagnostic tests, such as imaging studies or blood tests, to confirm the diagnosis and determine the underlying cause.
Treatment for Budd-Chiari Syndrome typically involves addressing the underlying cause of the condition, such as antibiotics for an infection or medication to treat an autoimmune disorder. In some cases, a procedure called thrombectomy may be necessary to remove a blood clot that is blocking the veins.
In severe cases, Budd-Chiari Syndrome can lead to complications such as stroke or heart failure, so it is important to seek medical attention promptly if symptoms persist or worsen over time. With timely and appropriate treatment, however, many people with this condition are able to recover and manage their symptoms effectively.
The symptoms of altitude sickness can vary in severity and may include:
* Headache
* Dizziness and lightheadedness
* Nausea and vomiting
* Fatigue and weakness
* Shortness of breath
* Coughing and chest tightness
* Swelling of the hands, feet, and face
In severe cases, altitude sickness can lead to more serious complications such as:
* High-altitude pulmonary edema (HAPE): fluid buildup in the lungs that can be life-threatening
* High-altitude cerebral edema (HACE): fluid buildup in the brain that can be life-threatening
To prevent altitude sickness, it is recommended to ascend gradually and give your body time to acclimate to the higher altitude. This can be done by spending a few days at a lower altitude before ascending to a higher altitude. It is also important to stay hydrated by drinking plenty of water and avoid alcohol and sedatives, which can increase the risk of altitude sickness.
If you experience any symptoms of altitude sickness, it is important to descend to a lower altitude as soon as possible. Medications such as acetazolamide (Diamox) can also be used to help prevent and treat altitude sickness. In severe cases, hospitalization may be necessary to receive oxygen therapy and other medical treatment.
The term splenomegaly is used to describe any condition that results in an increase in the size of the spleen, regardless of the underlying cause. This can be caused by a variety of factors, such as infection, inflammation, cancer, or genetic disorders.
Splenomegaly can be diagnosed through a physical examination, where the doctor may feel the enlarged spleen during an abdominal palpation. Imaging tests, such as ultrasound, computed tomography (CT) scans, or magnetic resonance imaging (MRI), may also be used to confirm the diagnosis and evaluate the extent of the splenomegaly.
Treatment for splenomegaly depends on the underlying cause. For example, infections such as malaria or mononucleosis are treated with antibiotics, while cancerous conditions may require surgical intervention or chemotherapy. In some cases, the spleen may need to be removed, a procedure known as splenectomy.
In conclusion, splenomegaly is an abnormal enlargement of the spleen that can be caused by various factors and requires prompt medical attention for proper diagnosis and treatment.
This type of leukemia is considered "chronic" because it progresses slowly over time, and it is "atypical" because the cancer cells do not fit into any of the standard subtypes of myeloid leukemia. It is also "BCR-ABL negative," meaning that there is no evidence of a specific genetic abnormality (the BCR-ABL fusion gene) that is present in other types of chronic myeloid leukemia.
This type of leukemia is relatively rare and tends to affect older adults. It can cause symptoms such as fatigue, fever, night sweats, and an enlarged spleen, and it can increase the risk of infections and bleeding. Treatment typically involves chemotherapy and, in some cases, bone marrow transplantation.
Polycythemia
Polycythemia vera
Twin anemia-polycythemia sequence
List of OMIM disorder codes
Reticulocyte production index
Scott Murphy (physician)
Hypoxemia
Exchange transfusion
Persistent fetal circulation
Phyllis George
Alan Aerts
Essential thrombocythemia
Givinostat
Diffusing capacity
Deep vein thrombosis
Mir-339 microRNA precursor family
EPAS1
Ruxolitinib
Fumarase deficiency
Deaths in March 2022
Hyperbilirubinemia in adults
Acute megakaryoblastic leukemia
Malar flush
Ranimustine
Ropeginterferon alfa-2b
Isaac Lipnitsky
Pipobroman
Primary myelofibrosis
Hemorheology
Pheochromocytoma
Polycythemia Vera - Polycythemia Vera | NHLBI, NIH
Polycythemia vera - About the Disease - Genetic and Rare Diseases Information Center
Polycythemia vera: MedlinePlus Genetics
ATSDR - Polycythemia Vera Investigation
Is Polycythemia a Cancer? Causes, Symptoms, Treatment
Treatment of polycythemia vera with hydroxyurea - PubMed
Chorea disclosing deterioration of polycythaemia vera | Postgraduate Medical Journal
Overcoming treatment challenges in myelofibrosis and polycythemia vera: the role of ruxolitinib - PubMed
Risk Factors of Polycythemia Vera | Hematology-Oncology Associates of CNY
NIH VideoCast - CC Grand Rounds: (1) A New Syndrome of Paraganglioma and Somatostatinoma Associated with Polycythemia: Clinical...
Polycythemia Vera | Nebraska Hematology Oncology - Cancer Care Treatment Blood Disorders Clinical Trials Lincoln Nebraska (NE)
Secondary Polycythemia Article
Cytoreductive treatment patterns among US veterans with polycythemia vera | BMC Cancer | Full Text
Polycythemia
Polycythemia Vera
Polycythemia vera | Diagnosaurus
Extramedullary haematopoiesis presenting with cardiac tamponade in a patient with polycythaemia vera - Fingerprint - Penn...
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TET2 gene: MedlinePlus Genetics
Developing polycythemia vera2
- In some of these families, the risk of developing polycythemia vera appears to have an autosomal dominant pattern of inheritance . (medlineplus.gov)
- Autosomal dominant inheritance means that one copy of an altered gene in each cell is sufficient to increase the risk of developing polycythemia vera, although the cause of this condition in familial cases is unknown. (medlineplus.gov)
People with polycythemia3
- Some people with polycythemia vera experience headaches, dizziness, ringing in the ears (tinnitus), impaired vision, or itchy skin. (medlineplus.gov)
- Although mutations in the TET2 gene have been found in approximately 16 percent of people with polycythemia vera, it is unclear what role these mutations play in the development of the condition. (medlineplus.gov)
- The arterial pressure is also normal in most people with polycythemia, although in about one third of them, the arterial pressure is elevated. (brainkart.com)
Absolute polycythemia3
- Chronic pulmonary disease (e.g., emphysema , which is abnormal distension of the lungs with air) and extreme obesity (which may severely impair pulmonary ventilation) result in absolute polycythemia (Pickwickian syndrome). (medicinenet.com)
- Absolute polycythemia, however, is when the exact cause is known and is called erythrocytosis. (medicinenet.com)
- On the other hand, the absolute polycythemia is characterized by absolute increase in the red blood cell mass circulating, as an outcome of a certain increase in the production of bone marrow. (meowlovers.com)
Transient polycythemia2
- Relative and transient polycythemia go away when the underlying condition that has caused it is treated. (medicinenet.com)
- The transient polycythemia is due to splenic contraction that injects concentrated red blood cells through the circulation in a brief reaction to epinephrine -a hormone that reacts to anger, fear, and stress. (meowlovers.com)
Secondary18
- Secondary polycythemia is a rare disease that involves the overproduction of red blood cells. (statpearls.com)
- This activity illustrates the evaluation and management of secondary polycythemia and highlights the role of the interprofessional team in improving care for patients with this condition. (statpearls.com)
- Identify the pathophysiology of secondary polycythemia. (statpearls.com)
- Describe the appropriate evaluation of secondary polycythemia. (statpearls.com)
- Outline management options available for secondary polycythemia. (statpearls.com)
- Summarize the importance of collaboration and communication amongst the interprofessional team to enhance care coordination for patients with secondary polycythemia. (statpearls.com)
- An elevated erythropoietin (EPO) level, usually as a secondary response to chronic hypoxemia, leads to secondary polycythemia. (statpearls.com)
- Secondary polycythemia is due to an increased level of EPO or other transcription factors that, in turn, lead to an increase in the production of RBC mass. (statpearls.com)
- Polycythemia vera: This neoplastic disorder is the result of increased erythroid progenitor cells as well as increased sensitivity to erythropoietin secondary to a mutation called JAK mutation. (statpearls.com)
- [1] Secondary polycythemia can also be classified as congenital or acquired depending upon when the individual developed the defect. (statpearls.com)
- This condition is called secondary polycythemia, and the red cell count commonly rises to 6 to 7 million/mm 3 , about 30 per cent above normal. (brainkart.com)
- A common type of secondary polycythemia, called physiologic polycythemia, occurs in natives who live ataltitudes of 14,000 to 17,000 feet, where the atmos-pheric oxygen is very low. (brainkart.com)
- Polycythemia can be caused by a bone marrow disorder caused polycythemia vera or from other causes outside the bone marrow called secondary polycythemia. (gboncology.com)
- Secondary polycythemia can be caused by conditions that cause low oxygen levels in the blood. (gboncology.com)
- The best treatment for secondary polycythemia is treating the underlying condition such as smoking cessation or the treatment of the sleep apnea. (gboncology.com)
- If none of the aforementioned secondary conditions is present, it's possible that the cause of the condition is polycythemia vera. (meowlovers.com)
- Secondary erythrocytosis such as those related with renal tumors should be taken into consideration during the diagnosis of polycythemia vera. (biomedcentral.com)
- Recent studies have demonstrated neutrophil overexpression of the polycythemia rubra vera-1 (PRV-1) gene in polycythemia vera (PV) but not in secondary or spurious polycythemia (SP). (uthscsa.edu)
Hematocrit5
- Polycythemia (also called polyglobulia) is a disease in which the hematocrit level (volume of red blood cells) and hemoglobin concentration are raised in the peripheral blood. (medicinenet.com)
- Polycythemia is considered when hematocrit levels are over 48% in women and 52% in men. (medicinenet.com)
- Absolute or true erythrocytosis differentiates from relative polycythemia, where the hematocrit is increased, but the red cell mass lies within the normal range. (statpearls.com)
- In addition to thosepeople who have physiologic polycythemia, others have a pathological condition known as polycythemiavera, in which the red blood cell count may be 7 to 8million/mm 3 and the hematocrit may be 60 to 70 per cent instead of the normal 40 to 45 per cent. (brainkart.com)
- In polycythemia vera, not only does the hematocrit increase, but the total blood volume also increases, on some occasions to almost twice normal. (brainkart.com)
Myeloproliferative disorder1
- [ 49 ] It most often occurs in patients with an underlying thrombotic diathesis, including in those who are pregnant or who have a tumor, a chronic inflammatory disease, a clotting disorder, an infection, or a myeloproliferative disorder , such as polycythemia vera or paroxysmal nocturnal hemoglobinuria . (medscape.com)
Symptoms10
- What are the signs and symptoms of polycythemia? (medicinenet.com)
- Symptoms of polycythemia occur gradually over time. (medicinenet.com)
- Many of the signs and symptoms of polycythemia vera are related to a shortage of oxygen in body tissues. (medlineplus.gov)
- Supplements can play a role in alleviating or eliminating the symptoms of Polycythemia Vera. (forbiddendoctor.com)
- Click here if you would like to watch an overview video of the diagnosis, presenting symptoms, disease course, and treatment options of polycythemia vera for patients, their caregivers, and their loved ones created by Dr. Ruben A. Mesa and Dr. Robyn M. Scherber of UT Health San Antonio, MD Anderson Cancer Center. (mpnfoundation.org)
- In this article we will discuss some more interesting facts about polycythemia in cats including the cause, symptoms, prognosis and treatment. (meowlovers.com)
- Due to the fact that the red blood cells influence all the systems of the body of a cat, the symptoms of polycythemia may become seemingly noticeable and somewhat unrelated symptoms. (meowlovers.com)
- The symptoms of polycythemia in cats may progress in a gradual manner and it might be hard to notice for several months. (meowlovers.com)
- Hyperviscosity is the cause of clinical symptoms in infants presumed to be symptomatic from polycythemia. (mhmedical.com)
- Half of these patients are symptomatic, although it is not at all certain whether their symptoms are caused by polycythemia. (mhmedical.com)
Bone marrow2
- Polycythemia vera (PV) as a primary condition is a type of clonal disorder of bone marrow stem cells which is often caused by a mutation in exon 12 of the janus kinase 2 ( JAK2 ) tyrosine kinase gene [ 4 , 5 ]. (biomedcentral.com)
- Polycythemia vera is a disease process resulting in excess production of blood cells and platelets in the bone marrow which actuates ischemic events in both the large and small vessels in the body. (fortunepublish.com)
Circulatory System1
- Regarded as an irregular increase in the number of red blood cells in the circulatory system, the polycythemia is a fairly serious blood condition. (meowlovers.com)
Primary myelofibrosis2
- Some diseases such as chronic myeloid leukemia (CML), chronic neutrophilic leukemia (CNL), and chronic eosinophilic leukemia (CEL) express primarily a myeloid phenotype, while in others such as polycythemia vera (PV), primary myelofibrosis (PMF), and essential thrombocytosis (ET), erythroid or megakaryocytic hyperplasia predominates. (mhmedical.com)
- Philadelphia-negative myeloproliferative neoplasms (MPN) are frequent and chronic myeloid malignancies including Polycythemia Vera (PV), essential thrombocythemia (ET), Primary Myelofibrosis (PMF) and Prefibrotic myelofibrosis (PreMF). (clinicaltrials.gov)
Genes are associated2
- Mutations in the JAK2 and TET2 genes are associated with polycythemia vera. (medlineplus.gov)
- Changes in expression levels of erythrocyte and immune-related genes are associated with high altitude polycythemia. (bvsalud.org)
Jakafi1
- Jakafi is a prescription medicine used to treat adults with polycythemia vera who have already taken a medicine called hydroxyurea (HU) and it did not work well enough or they could not tolerate it. (jakafi.com)
ATSDR2
- The Agency for Toxic Substances and Disease Registry (ATSDR) will update community members on the polycythemia vera (PV) research projects in the tri-county area of Schuylkill, Luzerne, and Carbon Counties, PA on September 20, 2012 in Tamaqua, PA. (cdc.gov)
- The federal Agency for Toxic Substances and Disease Registry (ATSDR) will hold a public meeting in the Tamaqua Area Auditorium at Tamaqua High School, 500 Penn St, Tamaqua, PA, on Saturday, October 24, 2009 from 10:00 to 11:30 a.m. to update area residents on recent efforts regarding polycythemia vera (PV). (cdc.gov)
Diagnosis1
- If you leave this without any diagnosis and treatment, polycythemia may lead to austere coronary signs, even heart failure. (meowlovers.com)
Phlebotomy1
- Treatment of polycythemia vera is removal of the extra blood in a process called phlebotomy. (gboncology.com)
Erythremia1
- Polycythemia Vera (Erythremia). (brainkart.com)
Abnormality1
- Congenital heart disorders, enzymatic or hemoglobin abnormality, excessive use of coal tar derivatives, and tumors can also cause polycythemia. (medicinenet.com)
Anemia1
- Twice as many babies die each year from Twin-to-Transfusion Syndrome (TTTS) and Twin Anemia Polycythemia Sequence (TAPS) than from Sudden Infant Death Syndrome (SIDS). (arizona.edu)
Neoplasms1
- The trigger for polycythemia vera (PV) and other myeloproliferative neoplasms (MPNs) isn't known. (mpnfoundation.org)
Occurs3
- Polycythemia vera typically develops in adulthood, around age 60, although in rare cases it occurs in children and young adults. (medlineplus.gov)
- Polycythemia occurs in 2-4% of the general newborn population. (mhmedical.com)
- Hyperviscosity without polycythemia occurs in 1% of normal (nonpolycythemic) newborns. (mhmedical.com)
Cardiac1
- Actually, the cardiac output in polycythemia is not far from normal, because these two factors more or less neutralize each other. (brainkart.com)
Genetic1
- In most cases, polycythemia is not innate (passed down through generations), yet it is genetic in a few cases. (medicinenet.com)
TET21
- Changes in the TET2 gene are also found in polycythemia cells. (medicinenet.com)
JAK22
- Typically, all individuals diagnosed with polycythemia have a mutated JAK2 gene. (medicinenet.com)
- JAK2 gene mutations seem to be particularly important for the development of polycythemia vera, as nearly all affected individuals have a mutation in this gene. (medlineplus.gov)
Ruxolitinib1
- Written by Neal Dave, PharmD, Texas Oncology Download Here Description: This PQI will review appropriate patient identification and management of Polycythemia Vera (PV) with the use of ruxolitinib therapy. (ncoda.org)
Patients2
- Patients with polycythemia may have other blood disorders as well. (medicinenet.com)
- However, some patients have hemoglobin that is too high called polycythemia. (gboncology.com)
Hemoglobin1
- Polycythemia is considered when hemoglobin levels are over 16.5 grams/dL in women and 18.5 grams/dL in men. (medicinenet.com)
Disease4
- Other complications of polycythemia vera include an enlarged spleen (splenomegaly), stomach ulcers, gout (a form of arthritis caused by a buildup of uric acid in the joints), heart disease, and cancer of blood-forming cells (leukemia). (medlineplus.gov)
- In these families, people seem to inherit an increased risk of polycythemia vera, not the disease itself. (medlineplus.gov)
- Polycythemia vera (PV) is a rare blood disease. (hoacny.com)
- We present an unusual case of a female with polycythemia vera who was diagnosed with ischemic gangrene in her extremities as a result of diabetic ketoacidosis, despite having only mildly elevated blood counts from her underlying disease. (fortunepublish.com)
Patient2
- Because of a history of polycythemia vera, the patient had been treated for the condition for 9 years. (biomedcentral.com)
- A 71-year-old man with polycythemia vera who was in the same ward as the index case-patient for 6 days acquired infection with H7N9 and pH1N1 viruses. (cdc.gov)
Occur1
- In rare cases, neonatal polycythemia may occur due to the transfusion of placental blood to the baby or chronic oxygen insufficiency of the fetus (intrauterine hypoxia ) due to placental inadequacy. (medicinenet.com)
Main1
- Further, she cause of this main form of polycythemia in cats isn't understood well, even up to this moment. (meowlovers.com)
Oxygen1
- Polycythemia is the result of increased demand for oxygen in the body. (medicinenet.com)
Condition3
- Polycythemia vera is a condition characterized by an increased number of red blood cells in the bloodstream. (medlineplus.gov)
- Polycythemia, derived from poly (many) and cythemia (cells in the blood), is a condition defined as an abnormal increase in the red blood cell (RBC) mass. (statpearls.com)
- The second one, the cause of the condition seen by the vet might be addressed, or in an instance of polycythemia vera, preventive treatment might be recommended. (meowlovers.com)
Relative2
- Relative polycythemia may be the result of abnormally high fluid intake or excessive loss of body fluid, for example, severe vomiting , diarrhea , or non-stop sweating . (medicinenet.com)
- The relative polycythemia may develop when a reduction in the plasma volume, commonly due to dehydration, may produce a fair increase in the red blood cells circulating. (meowlovers.com)
Stages1
- The treatment for polycythemia in cats may progress in two stages. (meowlovers.com)
Understood1
- As a chronic mountain sickness (CMS) with the highest incidence and the greatest harm, the pathogenesis of high altitude polycythemia (HAPC) is still not fully understood. (bvsalud.org)
Treatment options2
- What are treatment options for polycythemia? (medicinenet.com)
- We will review the various treatment options currently available for polycythemia vera. (fortunepublish.com)
Rare1
- In rare instances, polycythemia vera has been found to run in families. (medlineplus.gov)
Blood cells4
- A high concentration of red blood cells indicates polycythemia. (medicinenet.com)
- Polycythemia vera (PV) is a chronic, progressive myeloproliferative neoplasm (MPN) primarily characterized by an elevation of the red blood cells. (mpnfoundation.org)
- In medical terms, the overproduction of red blood cells in cats is known as polycythemia. (meowlovers.com)
- The first one, red blood cells may be removed directly from the bloodstream of the cat to treat the acute polycythemia. (meowlovers.com)
Body1
- Supplements aid the body in its own ability to address Polycythemia Vera. (forbiddendoctor.com)
Commonly1
- Also, the urine sample is commonly used in order to ascertain whether the polycythemia has started to affect the other organ systems or in ruling out some other diagnoses. (meowlovers.com)
Clinical1
- Clinical and hematological presentation of children and adolescents with polycythemia vera. (medlineplus.gov)
Types1
- What are different types of polycythemia? (medicinenet.com)
Medicine1
- Medicine Central , im.unboundmedicine.com/medicine/view/Diagnosaurus/114657/all/Polycythemia_vera. (unboundmedicine.com)