Title: Rare Case of Polycythemia Vera Presenting As Portal Vein and Splenic Vein Thrombosis – A Case Report
Authors: Revathi Potnuru, Ramana Murthy S.V, Padma Guduri
DOI: https://dx.doi.org/10.18535/jmscr/v8i2.03
Abstract
Polycythemia vera is one of the chronic myeloproliferative neoplasms. It is a clonal hematopoietic stem cell disorder in which phenotypically normal red cells, granulocytes, and platelets accumulate in the absence of a recognizable physiologic stimulus. It occurs in 2.5 per 100,000 persons, sparing no adult age group. Approximately one tenth cases present before age 40 years. Mutation in the JAK2V617F tyrosine kinase appears to play a central role in the pathogenesis of PV. It can cause venous or arterial thrombosis of any vessel, but cerebral, cardiac, and mesenteric vessels are most commonly involved. Here we report a case of 29 year old male patient, who was admitted with abdominal distension, icterus, pedal edema and was found to have portal vein and splenic vein thrombosis due to polycythemia vera on evaluation. It is important to identify such cases because it may cause death due to thrombosis or it may transform into AML.
References
- Tefferi, A., JAK2 mutations in polycythemia vera—molecular mechanisms and clinical applications. N Engl J Med, 2007;356:444-5.
- Pierre R, Vardiman JW, Imbert M, Brunning RD, Thiele J, Flandrin G. Polycythemia Vera. In: World Health Organization classification of tumors: pathology and genetics of tumors of haematopoitic and lymphoid tissues,2001;32-34.
- Spivak JL, Silver RT. The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal. Blood 2008;112:231.
- Tefferi A, Guglielmelli P, Larson DR, et al Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis. Blood 2014;124:2507.