PEDIATRICS Vol. 39 No. 5 May 1967, pp. 659-674
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PATHOLOGIC VARIANTS OF CONGENITAL HYPOGAMMAGLOBULINEMIA: AN ANALYSIS OF 3 PATIENTS DYING OF MEASLES

Allen I. Lipsey M.D.1, Myra J. Kahn 1, and Robert P. Bolande M.D.1

1 The Department of Pediatrics and Division of Pediatric Pathology, Institute of Pathology, Western Reserve University, Cleveland, Ohio

Three patients with abnormal thymus glands characterized principally by the absence of Hassall's corpuscles were presented. These patients do not clinically fall into either the ataxia-telangiectasia syndrome or the Swiss-type of agammaglobulinemia. This suggests that hypoplasia of the epithelial thymus, i.e., absence of Hassall's corpuscles, may be associated with a spectrum of lymphoid tissue histopatholgy and immunologic derangement, perhaps dependent on an additional lympho-immune primer. The immunologic disorders appear to have resulted in bizarre, widely disseminated lethal measles infections in each case.

Submitted on May 2, 1966
Accepted on December 8, 1966




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