ROBERT W. ENQUIST; JON P. GOCKERMAN; EDWIN H. JENIS; RAPHAEL L. WARKEL; DONALD E. DILLON
ENQUIST RW, GOCKERMAN JP, JENIS EH, WARKEL RL, DILLON DE. Type II Congenital Dyserythropoietic Anemia. Ann Intern Med. 1972;77:371-376. doi: 10.7326/0003-4819-77-3-371
Download citation file:
Published: Ann Intern Med. 1972;77(3):371-376.
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.
Learn more about subscription options.
Register Now for a free account.
Hematology/Oncology, Red Cell Disorders.
Results provided by:
Copyright © 2017 American College of Physicians. All Rights Reserved.
Print ISSN: 0003-4819 | Online ISSN: 1539-3704
Conditions of Use
This PDF is available to Subscribers Only