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Combined immunodeficiency presenting as the Letterer-Siwe syndrome

Authors
Journal
The Journal of Pediatrics
0022-3476
Publisher
Elsevier
Publication Date
Volume
85
Issue
4
Identifiers
DOI: 10.1016/s0022-3476(74)80446-4
Disciplines
  • Medicine

Abstract

Four infants with skin rash, hepatosplenomegaly, lymphocytosis, eosinophilia, and histiocytic infiltration of thelymph nodes and skin are described; in each of these infants an initial diagnosis of the Letterer-Siwe syndrome was made. Postmortem findings of thymic dysplasia and poorly differentiated, lymphopenic peripheral lymphoid tissue in each of the four infants, as well as antemortem clinical findings in one, established a diagnosis of severe combined immunodeficiency. From these and similar cases in the literature, we postulate that the Letterer-Siwe syndrome may not be an unusual presentation of combined immunodeficiency. Appropriate immunologic studies will help to differentiate the two disorders.

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