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Congenital DyskeratosisZinsser-Engman-Cole Syndrome With Thymic Dysplasia and Aplastic Anemia
Jorge A. Ortega, MD;
Virginia L. Swanson, MD;
Benjamin H. Landing, MD;
G. Denman Hammond, MD
Am J Dis Child. 1972;124(5):701-704.
Abstract
A child with the typical features of congenital dyskeratosis (Zinsser-Engman-Cole syndrome) and aplastic anemia, low serum -globulins, thymic dysplasia, lymphocyte-poor spleen and lymph nodes, and absence of primary follicles, is reported. The findings support an immunological defect and suggest that thymic dysplasia may be an integral feature of congenital dyskeratosis.
Author Affiliations
Los Angeles
From the Department of Pediatrics and Pathology, Childrens Hospital of Los Angeles, and the University of Southern California School of Medicine, Los Angeles.
Footnotes
Received for publication Jan 19, 1972; accepted April 21.
Reprint requests to 4650 Sunset Blvd, PO Box 54700, Los Angeles, Calif 90054 (Dr. Ortega).
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