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November 1972

Congenital Dyskeratosis: Zinsser-Engman-Cole Syndrome With Thymic Dysplasia and Aplastic Anemia

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.

Am J Dis Child. 1972;124(5):701-704. doi:10.1001/archpedi.1972.02110170079012

A child with the typical features of congenital dyskeratosis (Zinsser-Engman-Cole syndrome) and aplastic anemia, low serum γ-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.