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Type II Congenital Dyserythropoietic Anemia
- CPT ROBERT W. ENQUIST;
- MAJ JON P. GOCKERMAN;
- MAJ EDWIN H. JENIS;
- MAJ RAPHAEL L. WARKEL; and
- LTC DONALD E. DILLON
Abstract
Three children from a family with 10 members had congenital dyserythropoietic anemia, type II. These cases differ from those previously described in that Gaucher-like histiocytes were present in bone marrow aspirates from all affected children. The light and electron microscopic appearance of these cells resembled those described in chronic myelogenous leukemia and thalassemia. Using the endogenous carbon monoxide production as an index of total heme turnover and the erythrocyte 51Cr survival as an index of peripheral destruction, significant erythrocyte hemolysis was shown in the marrow. A recessive mode of inheritance is suggested, with the homozygous person having clinical disease and the heterozygous person having only serological abnormalities.
Article and Author Information
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▸From the Department of Medicine, Division of Hematology, and Pathology Service, Walter Reed General Hospital, and the Walter Reed Army Institute of Research, Washington, D.C.
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This material has been reviewed by the Office of the Surgeon General, Department of the Army, and there is no objection to its presentation or publication. The review does not imply any endorsement of the opinions advanced or any recommendation of such products as may be named.
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▸Requests for reprints should be addressed to CPT Robert W. Enquist, MC, USAR, Department of Medicine, Walter Reed General Hospital, Washington, D.C. 20012.
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- Received March 23, 1972.
- Accepted June 8, 1972.
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