[Splenectomy in paroxysmal nocturnal hemoglobinuria with pancytopenia].
Two patients with long known paroxysmal nocturnal haemoglobinuria (PNH) were hospitalized for progressive severe pancytopenia or thrombocytopenia. Clinical and laboratory findings revealed a hematologic complication related to the course of PNH. In view of normocellular bone marrow hematopoiesis and documented increased splenic sequestration of RBC, functional hypersplenism was diagnosed. Selective splenectomy confirmed this hypothesis and was followed by long-standing remission of pancytopenia for 10 or more years in both patients.
D000368:Aged; D005260:Female; D006410:Hematopoiesis; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D006971:Hypersplenism / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010198:Pancytopenia / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D013156:Splenectomy
Splenektomie bei paroxysmaler nächtlicher Hämoglobinurie mit Panzytopenie.