JAK2V617F positive polycythemia vera with paroxysmal nocturnal hemoglobinuria and visceral thromboses: a case report and review of the literature

نویسندگان

چکیده

Abstract Background Polycythemia vera (PV) is characterized by red cell mass expansion in the peripheral blood and can be complicated with thrombosis, bleeding, evolution to acute myeloid leukemia (AML) or a fibrotic phase. Paroxysmal nocturnal hemoglobinuria (PNH) an acquired clonal haematopoietic stem disorder associated chronic intravascular hemolysis, venous defective hematopoiesis, frequent episodes of infection and, rarely, leukemic transformation. Herein, we report interesting case patient coexistence PNH clones JAK2V617F positive PV, unusual thromboses without hemolysis. Case presentation A 51-year-old woman presented increased levels hematocrit, multiple liver, spleen, left kidney infarctions ascites; further investigation revealed -positive polycythemia presence significant population (more than 90% CD55– CD59– cells among both granulocytes cells). Interestingly, has experienced severe thrombotic events any signs symptoms Conclusions This raises questions over uncharted aspects etiopathogenesis its potential association myeloproliferative neoplasms (MPN) highlights difficulty diagnosing managing patients more one potentially thrombophilic conditions, especially established thromboses.

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ژورنال

عنوان ژورنال: Thrombosis Journal

سال: 2021

ISSN: ['1477-9560']

DOI: https://doi.org/10.1186/s12959-021-00269-8