Splenectomy for massive splenic infarction unmasks paroxysmal nocturnal hemoglobinuria.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder characterized by pancytopenia, hemolysis, and thrombosis. Abdominal vein thrombosis is a life-threatening manifestation of this disease. We present a patient with complete spleen necrosis due to thrombosis of the splenic vessels. After splenectomy, other causes of thrombophilia were excluded and the diagnosis of PNH was established. The patient was put on anticoagulation but despite the prophylactic international normalized ratio maintained over the last 18 months of follow-up, he had another episode of intrahepatic thrombosis which was treated with tissue plasminogen activator thrombolysis.
Tsatalas
Costas
C
Department of Hematology, School of Medicine, Democritus University of Thrace, Alexandroupolis, Greece. ktsatala@med.duth.gr
Margaritis
Dimitris
D
Pantelidou
Despina
D
Kotsianidis
Ioannis
I
Karayiannakis
Anastasios J
AJ
Spanoudakis
Emanuel
E
Kartasis
Zafiris
Z
Kaloutsi
Vasiliki
V
Polychronidis
Alexandros
A
Manavis
Ioannis
I
Bourikas
George
G
D000328:Adult; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008297:Male; D013156:Splenectomy; D013159:Splenic Infarction / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D014057:Tomography, X-Ray Computed