Spontaneous splenic rupture in polycythemia vera

Abstract

Atraumatic spontaneous splenic rupture is rare and occurs in the setting of spleen pathology from a myriad of causes, including myeloproliferative disorders. Only four previously reported cases of atraumatic spontaneous splenic rupture associated with polycythemia vera exist. We report the fifth case, which also is the first with severe thrombocytosis requiring multiple plateletpheresis despite medical therapy and excellent long-term control. In addition to reporting the fifth case of spontaneous splenic rupture in a polycythemia vera patient, we also review the risks associated with splenectomy in patients with polycythemia vera and therapies that can prevent spontaneous splenic rupture.

A Rare Case of Triple-Negative Essential Thrombocythemia in a Young Postsplenectomy Patient: A Diagnostic Challenge

“…Atraumatic spontaneous splenic rupture is rarely documented to be associated with myeloproliferative neoplasms especially with polycythemia vera (PV). To date, there are only five cases in world literature of atraumatic spontaneous splenic rupture associated with PV [13–17]. All five cases presented with splenomegaly and hemodynamic instability and required an exploratory laparotomy at which time a splenectomy was performed.…”