A Case of Aplastic Anemia-PNH Syndrome

Abstract

A 34-year-old man was admitted because of pallor, fatigability and bleeding tendency in March, 1970. After admission, sternal marrow aspirations showed normocellularity with erythroid hyperplasia and iliac crest aspirations were hypocellular. Pancytopenia persisted. A reticulocyte count was 2.0% and red cell survival with ⁵¹Cr was 19 days (T 1/2). Both Ham and sugar-water tests were negative. Neither hemoglobinuria nor hemosiderinuria was noticed throughout the hospital stay at least up to July, 1973. Treatment with adrenocortico steroid and anabolic steroid hormones and bone marrow transfusions brought no hematological improvement. In February, 1973, 3 years later from the onset of the disease, Ham and sugar-water tests turned to be positive without hemoglobinuria, hemolytic crisis, nor thrombosis during the 3 years. However, apparent shortened red cell life span and combined ferrokinetic pattern of aplastic and hemolytic anemia were again detected on admission in March 1973.
It is likely that this case was on transitional phase from so-called “atypical” aplastic anemia to PNH and had signs of apparent hemolysis in spite of the absence of hemoglobinuria.