Is There a Role for Bone Marrow Biopsy In The Diagnosis of Masked Polycythemia Vera with Splanchnic Vein Thrombosis?

[摘要] Background: Philadelphia-negative chronic myeloproliferative neoplasms are the most common underlying prothrombotic disorders found in patients with splanchnic vein thromboses. A sizeable proportion of patients with splanchnic vein thromboses carry a Jak2-V617F mutation, despite the absence of overt signs of myeloproliferative neoplasm. Case Report: We report a case of a 46-year-old male presenting with splenomegaly and anemia. Subsequently, due to the appearance of abdominal pain, thrombosis of the portal vein, intra-hepatic portal branches and splenic vein were found. The simultaneous presence of splenomegaly and splanchnic vein thromboses prompted a search for an underlying myeloproliferative neoplasm and a diagnosis of splanchnic vein thromboses from a “masked polycythemia vera” was posed. Conclusion: This uncommon case highlights the importance of an early myeloproliferative neoplasms screening when a splanchnic vein thromboses is diagnosed.

[学科分类] 生理学

[关键词] Myeloproliferative Disorders;Polycythemia Vera;Portal Vein;Splenic Vein;Splenomegaly;Thrombosis.