Recurrent Infections, Episodic Lymphopenia and Impaired Cellular Immunity

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Abstract
Two siblings, a boy and a girl, died in childhood with an illness characterized by recurrent infections, eczema and episodes of lymphopenia. Post-mortem examinations in both showed depletion of small lymphocytes from thymus-dependent areas of peripheral lymphoid organs. Immunologic studies carried out in the boy revealed dysgammaglobulinemia, impaired cellular immunity, and absent immunologic memory. By means of a 51Cr-release assay, it was possible to demonstrate the presence of a complement-dependent, lymphocytotoxic factor in the boy's serum during an episode of lymphopenia. It is suggested that these patients with "immunologic amnesia" resemble laboratory animals treated with antilymphocyte serum.