Essential thrombocythemiaThrombosis of the hepatic veinCause of Budd-Chiari sPeople with polycythemia veraPortal vein thromObstructionHypercoagulableOcclusionParoxysmalMyelofibrosisVenousHematocritJAK2AscitesMyeloproliferative disordersUncommonMutationDisordersDisorderIdiopathicPulmonaryDiagnosisSecondaryRight atriumMyelodysplasticSerumSigns and symptomsOccurPatientsPrimaryChronic myelogenBoneCancersVesselsIncidenceSymptomsAbdominal painCommonlyClinicalCasesAdults
Essential thrombocythemia4
- Driver mutations among very young patients with (A) essential thrombocythemia (n=206) or (B) polycythemia vera (n=55). (capsulehealth.one)
- Characteristics and outcomes of patients with essential thrombocythemia or polycythemia vera diagnosed before 20 years of age: a systematic review. (capsulehealth.one)
- INTRODUCTION - An overview of the four classic myeloproliferative neoplasms (MPN): polycythemia vera, essential thrombocythemia, primary myelofibrosis, and chronic myeloid leukemia will be presented here. (medilib.ir)
- MPNs include polycythemia vera, essential thrombocythemia, chronic myeloid leukemia (CML), primary myelofibrosis, chronic neutrophilic leukemia, and other less well defined entities such as chronic eosinophilic leukemia, not otherwise categorized. (medilib.ir)
Thrombosis of the hepatic vein2
- Primary Budd-Chiari syndrome occurs due to thrombosis of the hepatic vein. (wikipedia.org)
- Inherited disorders of hypercoagulability may lead to thrombosis of the hepatic vein and Budd-Chiari syndrome. (wikipedia.org)
Cause of Budd-Chiari s2
- The cause of Budd-Chiari syndrome can be found in more than 80% of patients. (wikipedia.org)
- We conclude that right atrial myxoma is a rare cause of Budd-Chiari syndrome. (fortunejournals.com)
People with polycythemia vera1
- The V617F mutation is found in approximately 96 percent of people with polycythemia vera. (medlineplus.gov)
Portal vein throm1
- Budd-Chiari syndrome (BCS) and portal vein thrombosis have been reported to be associated with JAK2V617F-positive hematopoiesis. (uludag.edu.tr)
Obstruction8
- Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of the hepatic venous outflow and is characterized by hepatomegaly, ascites, and abdominal pain. (medscape.com)
- Budd-Chiari syndrome should be considered separate from veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome, which is characterized by toxin-induced, nonthrombotic obstruction of prehepatic veins (see the images below). (medscape.com)
- Any obstruction of the venous vasculature of the liver is referred to as Budd-Chiari syndrome, from the venules to the right atrium. (wikipedia.org)
- Budd-Chiari Syndrome Budd-Chiari syndrome is obstruction of hepatic venous outflow that originates anywhere from the small hepatic veins inside the liver to the inferior vena cava and right atrium. (msdmanuals.com)
- Budd-Chiari syndrome (BCS) is a rare condition marked by a number of symptoms due to hepatic venous obstruction. (fortunejournals.com)
- Budd-Chiari syndrome is an uncommon disorder resulting from obstruction of the large hepatic veins or inferior vena cava at the suprahepatic level. (fortunejournals.com)
- Alternatively, this syndrome is characterized by occlusion due to hepatic vein thrombosis or mechanical venous obstruction. (fortunejournals.com)
- Budd-Chiari syndrome is characterized by obstruction of hepatic venous flow at the level of right atrium, inferior vena cava, large hepatic veins, or hepatic venules [1]. (fortunejournals.com)
Hypercoagulable4
- Other acquired hypercoagulable disorders that may result in Budd-Chiari syndrome include antiphospholipid syndrome and paroxysmal nocturnal hemoglobinuria, which are responsible for 10-12% and 7-12% of Budd-Chiari syndrome cases, respectively. (wikipedia.org)
- Budd-Chiari syndrome may be the presenting sign of these hypercoagulable disorders. (wikipedia.org)
- Several factors predispose to the development of Budd-Chiari syndrome, including hypercoagulable, inherited, and acquired conditions, as well as a variety of other causes, can be identified in approximately 75% of patients. (fortunejournals.com)
- Because of their hypercoagulable state and risk of hemorrhage, patients with polycythemia vera who present with an acute myocardial infarction pose a challenge to physicians. (bvsalud.org)
Occlusion3
- Overt Budd-Chiari syndrome generally requires the occlusion of at least 2 hepatic veins. (medscape.com)
- Budd-Chiari syndrome (hepatic vein thrombosis) refers to occlusion of the hepatic vein by a thrombus, often resulting in death. (brainkart.com)
- This case report describes the presentation and treatment of a Hispanic patient with JAK2 V617F-negative primary polycythemia who developed cardiac arrest and ST-segment elevation myocardial infarction owing to complete occlusion of the left anterior descending artery as well as bleeding complications and postmyocardial pericarditis. (bvsalud.org)
Paroxysmal3
- [ 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)
- People who have paroxysmal nocturnal hemoglobinuria (PNH) appear to be especially at risk for Budd-Chiari syndrome, more than other forms of thrombophilia: up to 39% develop venous thromboses, and 12% may acquire Budd-Chiari. (wikipedia.org)
- While a few cases are idiopathic, more often there is an underlying process predisposing for the thrombosis e.g., polycythe-mia vera, pregnancy, oral contraceptives, paroxysmal nocturnal hemoglobinuria, or hepatocellular carcinoma. (brainkart.com)
Myelofibrosis1
- Ruxolitinib, a JAK1/2 inhibitor licensed for polycythemia vera and myelofibrosis, demonstrated efficacy in regulating hematocrit levels, lowering spleen volume, and relieving MPN-related symptoms. (bvsalud.org)
Venous2
- Budd-Chiari syndrome is also seen in tuberculosis, congenital venous webs and occasionally in inferior vena caval stenosis. (wikipedia.org)
- up to 39% develop venous thromboses, [17] and 12% may acquire Budd-Chiari. (wikipedia.org)
Hematocrit1
- In polycythemia vera, in contrast to secondary erythrocytosis, the red cell mass increase is often initially masked by an increase in the plasma volume that leaves the hematocrit in the normal range. (msdmanuals.com)
JAK23
- Somatic mutations in the JAK2 gene are associated with polycythemia vera, a disorder characterized by uncontrolled blood cell production. (medlineplus.gov)
- More than ninety percent of individuals with polycythemia vera have a variant (mutation) inside the JAK2 gene. (usa-good.com)
- Thrombotic and bleeding complications are major causes of morbidity and mortality in patients with polycythemia vera, who predominantly present with an alteration in the JAK2 gene. (bvsalud.org)
Ascites1
- The fulminant syndrome presents early with encephalopathy and ascites. (wikipedia.org)
Myeloproliferative disorders1
- Cytogenetic studies detect the presence or absence of the Philadelphia chromosome and help to differentiate myeloproliferative disorders from myelodysplastic syndrome. (medscape.com)
Uncommon1
- Polycythemia vera is uncommon. (usa-good.com)
Mutation1
- Budd-Chiari syndrome, which results from a blocked vein in the liver, can also be associated with the V617F mutation when it is caused by an underlying bone marrow disorder. (medlineplus.gov)
Disorders1
- Many individuals with polycythemia vera slowly development a selection of trendy, nonspecific symptoms that are not unusual to many disorders which includes headaches, fatigue, weak spot, dizziness, immoderate sweating especially at night, and itchy pores and skin that, in extreme instances, can be worse after getting showered or a heat bath. (usa-good.com)
Disorder2
- But right hospital treatment can help ease signs, signs and headaches of this disorder.Polycythemia vera is an extraordinary, persistent sickness related to the overproduction of blood cells within the bone marrow (myeloproliferative). (usa-good.com)
- Polycythemia is a clinical disorder characterized by a relative increase in the number of red blood cells in the peripheral blood. (symptoma.com)
Idiopathic1
- These cases are known as idiopathic Budd-Chiari syndrome. (wikipedia.org)
Pulmonary2
- Because in chronic pulmonary disease both hypoxemia and polycythemia are simultaneously present, their individual effects on the pulmonary circulation have been difficult to define. (symptoma.com)
- Chronic pulmonary disease (e.g., emphysema-abnormal distension of the lungs with air) may produce chronic hypoxemia (reduced oxygen tension in the blood) and lead to absolute polycythemia. (symptoma.com)
Diagnosis2
- The prognosis is poor in patients with Budd-Chiari syndrome who remain untreated, with death resulting from progressive liver failure in 3 months to 3 years from the time of the diagnosis. (medscape.com)
- See "Clinical manifestations and diagnosis of polycythemia vera" . (medilib.ir)
Secondary1
- Secondary Budd-Chiari syndrome, which is very rare compared to the primary variant, is due to compression of the hepatic vein by an outside structure (such as a tumor or polycystic kidney disease). (wikipedia.org)
Right atrium1
- Herein, our authors present a case of a 40-year-old female patient with Budd-Chiari syndrome in whom the right atrium myxoma was manifested with worsening of clinical symptoms despite medical treatment therapy. (fortunejournals.com)
Myelodysplastic1
- Myelodysplastic syndromes (MDS) are characterized by cellular dysplasia, variable degrees of peripheral blood cytopenias, and bone marrow hyperplasia (or less often, hypoplasia) [ 5 ]. (medilib.ir)
Serum1
- In polycythemia vera, RBC production proceeds independently of the serum erythropoietin level, which is usually low but can be normal. (msdmanuals.com)
Signs and symptoms2
- Many of the signs and symptoms of polycythemia vera are related to a lack of oxygen in body tissues. (medlineplus.gov)
- The signs and symptoms of polycythemia vera generally broaden slowly over a few years. (usa-good.com)
Occur2
- The long-term risks of polycythemia vera (PV) include leukemic and fibrotic transformation, which occur in fewer than 5% and 10% of patients, respectively, at 10 years. (medscape.com)
- 80% of all Budd-Chiari syndrome (BCS) cases occur in women. (unboundmedicine.com)
Patients2
- The majority of patients have a slower-onset form of Budd-Chiari syndrome. (wikipedia.org)
- We explored whether JAK2V617F was present in ECs in the vessels of polycythemia vera (PV) patients with BCS using laser capture microdissection followed by nested polymerase chain reaction or reverse-transcribed polymerase chain reaction. (uludag.edu.tr)
Primary1
- In primary polycythemia, inherent or acquired problems with red blood cell production lead to polycythemia. (symptoma.com)
Chronic myelogen1
- Bone marrow fibrosis is also detected in the spent phase of chronic myelogenous leukemia and polycythemia vera. (medscape.com)
Bone1
- Polycythemia Vera Polycythemia vera is a myeloproliferative neoplasm of the blood-producing cells of the bone marrow that results in overproduction of all types of blood cells. (merckmanuals.com)
Cancers2
- Polycythemia vera (PV) is an unprecedented type of blood in most cancers wherein your body produces too many pink blood cells. (usa-good.com)
- Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a type of blood for most cancers. (usa-good.com)
Vessels2
- Klippel-Trenaunay syndrome is a condition that affects the development of blood vessels, soft tissues (such as skin and muscles), and bones. (nih.gov)
- n\nOther complications of Klippel-Trenaunay syndrome can include a type of skin infection called cellulitis, swelling caused by a buildup of fluid (lymphedema), and internal bleeding from abnormal blood vessels. (nih.gov)
Incidence1
- Trends in the incidence of polycythemia vera among Olmsted County, Minnesota residents, 1935-1989. (msdmanuals.com)
Symptoms1
- If a person has developed polycythemia as a result of liver cancer, kidney cancer, or other erythropoietin-secreting tumors, then symptoms include weight loss, abdominal fullness or pain and jaundice. (symptoma.com)
Abdominal pain1
- The acute syndrome presents with rapidly progressive severe upper abdominal pain, yellow discoloration of the skin and whites of the eyes, liver enlargement, enlargement of the spleen, fluid accumulation within the peritoneal cavity, elevated liver enzymes, and eventually encephalopathy. (wikipedia.org)
Commonly1
- Budd-Chiari syndrome is most commonly diagnosed using ultrasound studies of the abdomen and retrograde angiography. (wikipedia.org)
Clinical2
- Medicine Central , im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/1688517/0.2/Budd_Chiari_Syndrome. (unboundmedicine.com)
- Polycythemia vera changed into first suggested inside the clinical literature in 1892. (usa-good.com)
Cases1
- An important non-genetic risk factor is the use of estrogen-containing forms of hormonal contraception, which is implicated in 22% of cases of Budd-Chiari syndrome. (wikipedia.org)
Adults1
- Budd-Chiari syndrome is a very rare condition, affecting one in a million adults. (wikipedia.org)