Common complication seen in polycythemia vera is:
Correct Answer: Myelofibrosis
Description: Ref: Robbins Pathologic Basis of Disease, 8th edition, Pg: & Harrison s online 18th edition.Explanation:Polycythemia Vera (PCV)Increased marrow production of red cells, granulocytes, and platelets [panmyelosis),PCV is associated with activating point mutations in the tyrosine kinase JAK2.Representative iron Values in Normal Subjects. Patients with Hemochromatosis, and Patients withAlcoholic Liver DiseaseDeterminationNormalSymptomaticHemochromatosisAlcoholic Liver DiseasePlasma iron. mmol/L (mg/dL)9-27(50-150)32-54 (180-300)|Total iron-binding capacity.45-66(250-370)N 36-54 (200-300) (250-370)N 45-66 (250-370)Transferrin saturation, percent22-4650-100N or | 27-60Serum ferritin, mg/L |||| 900-6000|| 10-500Men20-250 Women15-150 Liver iron, mg/g dry wt300-1400|||| 6000-18.000|| 300-2000Hepatic iron index<1.0>2<2* JAK2 participates in the JAK/STAT pathway. which lies downstream of multiple hematopoietic growth factor receptors. including the erythropoietin receptor.MorphologyThe marrow is hypercellular. but some residual fat is usually present.At diagnosis, a moderate to marked increase in reticulin fibers is seen in about 10% of marrows.The peripheral blood often contains increased basophils and abnormally large platelets.Late in the course. PCV often progresses to a 'spent phase' characterized by extensive marrow fibrosis that displaces hematopoietic cells. This is accompanied by increased extramedullary hematopoiesis in the spleen and liver, often leading to prominent organomegahTransformation to AML occurs in about 2% of patients.Clinical FeaturesIt appears insidiously, usually in adults of late middle age.Most symptoms are related to the increased red cell mass, hematocrit and total blood volume.Patients are plethoric and cyanotic due to stagnation and deoxygenation of blood in peripheral vessels.Headache, dizziness, hypertension, and gastrointestinal symptoms are common.Intense pruritus and peptic ulceration may occur, both possibly resulting from the release of histamine from basophils.High cell turnover gives rise to hyperuricemia; Symptomatic gout in 5% -111%The abnormal blood flow and platelet function lead major bleeding and thrombotic episodes. About 25% present with deep venous thrombosis, myocardial infarction, or stroke.Thromboses sometimes also occur in the hepatic veins (producing Budd-Chiari syndrome) and the portal and mesenteric veins i leading to bowel infarction).M inor hemorrhages (epistaxis, bleeding gums) are common.The hemoglobin concentration - 14 to 28 gm/ dL. (Hematocrit >60%)The white cell count ranges from 12,000 to 50.000 cells/mm3. and the platelet count is often greater than 500.0(H) platelets/mm3.Giant platelets with defective aggregation.It is marked by the appearance of obliterative fibrosis in the bone marrow (myelofibrosis) and extensive extramedullary hematopoiesis. principally in the spleen, which enlarges greatly.ComplicationsDue to increase in blood viscosity associated with red cell mass elevationPeptic ulcer disease may be due to Helicobacter pylori and thePruritus due to basophil activation by JAK2 V617FSplenic infarctionMyelofibrosisExtramedullary hematopoiesis (Hepato- splenomegaly)Transfusion-dependent anemiaStem cell failure.Portal hypertensionProgressive cachexia.Acute Myelogenous Leukemia (AML)ErythromelalgUiIt is a curious syndrome of unknown etiology associated with thrombocytosisPrimarily involves the lower extremities Manifested by erythema, warmth, and pain of the affected appendageRarely, can also cause digital infarction.It is usually responsive to salicylates.'Ocular migraine' seen in PV patients, is a variant of erythromelalgia.
Category:
Medicine
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now