Acquired pure red cell aplasia associated with chronic lymphocytic leukemia

Pure red cell aplasia (PRCA) represents selective failure of erythropoiesis in the absence of abnormalities in leukopoiesis or thrombocytopoiesis. Acquired PRCA has occurred uncommonly in chronic lymphocytic leukemia (CLL). We report six patients with non-T-cell CLL in which nine episodes of PRCA were observed. In six of the nine episodes, the patients had received recent oral alkylating agents; conversely, recovery from aplasia occurred in some while on alternate alkylator therapy and two patients had no pretreatment. Spontaneous occurrence and recovery, therefore, could not be excluded. Onset of PRCA may be suggested by macrocytosis, but discontinuance of therapy at its development may not prevent PRCA if, indeed, therapy is one of the causes. Supportive therapy or alkylators and prednisone permitted recovery without mortality in all six patients. The interval between onset of CLL and the recognition of first PRCA episode varied from one month to nine years (mean, 5.3 years); the duration of PRCA ranged from three to nine months (mean, 4.7 months). In addition to previously better understood causes of anemia in CLL, the uncommon occurrence of PRCA is an additional consideration in the differential diagnosis of sudden anemia in CLL.