IMMUNOPATHOLOGY OF THE X-LINKED LYMPHOPROLIFERATIVE SYNDROME NLM AIDSLINE Important note: Information in this article was accurate in 1982. The state of the art may have changed since the publication date.

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IMMUNOPATHOLOGY OF THE X-LINKED LYMPHOPROLIFERATIVE SYNDROME

Hamatol Bluttransfus; 26:207-214 1981. Unique Identifier : AIDSLINE ICDB/81632513
Purtilo DT; Dept. Pathology, Univ. Massachusetts Medical Center, Worcester,; MA, 01605

Abstract: Immunopathologic studies of affected individuals and carrier females from 25 kindreds exhibiting the X-linked lymphoproliferative syndrome (XLP) were carried out. XLP is a combined, variable immune deficiency with four phenotypes, all apparently triggered by Epstein-Barr virus (EBV) infection: (1) fatal infectious mononucleosis (IM), (2) chronic IM progressive to malignant lymphoma, (3) acute IM progressive to acquired agammaglobulinemia, and (4) malignant lymphoma. The clinical and pathological manifestations of XLP mimic the graft-versus-host reaction. Cytogenetic studies have revealed a spectrum from no appreciable defects to extensive aneuploidy in both peripheral lymphocytes and early passage lymphocytic lymphosarcoma cells; both affected and carrier individuals show a propensity to chromosome damage. Only weakly reactive anti-EBV antibodies are found, and anti-EBV nuclear antigen is lacking in affected individuals. Specific histopathology of the four phenotypes is discussed. These findings support the hypothesis that in XLP an immune deficiency toward EBV permits chronic and fatal lymphoproliferative responses to EBV infection. If this hypothesis is accurate, genetic counseling, providing of high titer gamma globulin, and antiviral therapy may be of prophylactic use. (58 Refs)
Keywords: MEETING PAPER

KWDmeetingpaper
820228
M8220006

Copyright © 1982 - National Library of Medicine. Reproduced under license with the National Library of Medicine, Bethesda, MD.

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