Pure red cell aplasia
Richard A. Savage, M.D.
Department of Laboratory Hematology and Blood Banking
Significant progress has recently been made in the characterization of the preleukemic phase of acute nonlymphocytic leukemia (ANLL). In addition to the well-defined syndromes which have been found to precede ANLL, a number of less well-defined and poorly understood preleukemic states have been described.1 The well-defined syndromes associated with increased risk of ANLL include exposure to myelotoxic agents such as chloramphenicol,2, 3 benzene,4, 5 phenylbutazone,6 and arsenic;7 exposure to ionizing radiation;8 several chromosomal abnormalities including Down’s syndrome,8 Klinefelter’s syndrome,9 and Turner’s syndrome;10 Fanconi’s congenital marrow hypoplasia;11 congenital agranulocytosis;12 idiopathic aplastic anemia;13 ataxia telangiectasia;14 and paroxysmal nocturnal hemoglobinuria.15
The syndrome of acquired purered cell aplasia (PRCA) with or without antecedent exposure to marrow toxins has only rarely been reported as a preleukemic phase of ANLL. PRCA may be defined as a normocytic, normochromic anemia without reticulocytes, but with normal leukocytes and platelets in the peripheral blood; normal marrow granulocytic and megakaryocytic elements with a virtual absence of erythroblastic elements, and no evidence of extramedullary hematopoiesis.16 The following case illustrates idiopathic PRCA as a preleukemic manifestation of ANLL.
A 56-year-old man was found to be anemic at another hospital in December 1970 following surgery for a renal calculus. A bone marrow aspirate was interpreted as showing aplasia. No history of exposure to organic toxins, insecticides, or chloramphenicol could be elicited. Empiric trials of prednisone and pyridoxine failed to improve the anemia, and herequired frequent transfusions to maintain adequate hemoglobin and hematocrit. On admission to the Cleveland Clinic Hospital he was . . .