A case of paroxysmal nocturnal hemoglobinuria presenting with intra-abdominal bleeding due to splenic rupture, developing renal infarct.
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.
Uzun
S
S
Division of General Internal Medicine, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey. drsamiuzun@gmail.com
Alpay
N
N
Ozturk
G B
GB
Saka
B
B
Yenerel
M
M
Erten
N
N
Karan
M A
MA
Taşcioglu
C
C
D015746:Abdominal Pain / Q000097:blood / Q000000981:diagnostic imaging; D000328:Adult; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications / Q000175:diagnosis*; D006470:Hemorrhage / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D007238:Infarction / Q000000981:diagnostic imaging / Q000209:etiology*; D007668:Kidney / Q000098:blood supply*; D012422:Rupture, Spontaneous / Q000150:complications / Q000209:etiology; D013159:Splenic Infarction / Q000150:complications / Q000209:etiology; D013161:Splenic Rupture / Q000150:complications / Q000209:etiology*; D014057:Tomography, X-Ray Computed