Uzun S, Alpay N, Ozturk G B, Saka B, Yenerel M, Erten N, Karan M A, Taşcioglu C
Division of General Internal Medicine, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Int J Lab Hematol. 2008 Jun;30(3):248-53. doi: 10.1111/j.1751-553X.2007.00949.x.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder characterized by intravascular hemolysis, hemoglobinuria, and thrombosis. Thrombotic attacks are life threatening and are responsible for nearly 50% of PNH-related deaths. Compared with thrombotic events, bleeding related to thrombocytopenia in PNH is quite rare. This report describes an atypical clinical presentation with problems in the diagnosis and management of a woman who presented with a splenic infarct followed by massive intra-abdominal bleeding due to splenic rupture. She also developed a renal infarct during hospitalization after diagnosis.