Immunologic and clinical effects of repeated blood exchange in familial erythrophagocytic lymphohistiocytosis. NLM AIDSLINE Important note: Information in this article was accurate in 1983. The state of the art may have changed since the publication date.

Click here to return to AIDSLINE main menu
DonateNow
Print this Article


Immunologic and clinical effects of repeated blood exchange in familial erythrophagocytic lymphohistiocytosis.

Blood. 1982 Oct;60(4):814-21. Unique Identifier : AIDSLINE MED/83000805
Ladisch S; Ho W; Matheson D; Pilkington R; Hartman G

Abstract: Depressed cellular immune function and increased susceptibility to infection characterize familial erythrophagocytic lymphohistiocytosis (FEL), a usually fatal autosomal recessive disease. One component of the immunodeficiency is plasma-mediated inhibition of lymphocyte proliferation. We have tested whether repeated plasma or blood exchange would decrease plasma inhibitory activity and improve cellular immune function in FEL. Following this treatment, reduction in plasma inhibitory activity, reversal of depressed antigen-specific lymphocyte proliferative responses and monocyte antibody-dependent cytotoxic function in vitro, and clinical improvement were complete in two and partial in one of three patients studied. Relapse, which was ultimately fatal, was associated with recurrence of the immune defects. These findings suggest that cellular immunodeficiency in FEL is acquired and possibly related to circulating immunosuppressive activity, the removal of which is associated with transient immunologic and clinical recovery.
Keywords: Antibody-Dependent Cell Cytotoxicity Candidiasis/ETIOLOGY Case Report Child, Preschool Exchange Transfusion, Whole Blood Female Human Infant Lymphocyte Transformation Male Monocytes/IMMUNOLOGY *Plasma Exchange Reticuloendotheliosis/BLOOD/COMPLICATIONS/*THERAPY Support, U.S. Gov't, P.H.S. JOURNAL ARTICLE

KWDantibody-dependentcellcytotoxicitycandidiasis/etiologycasereportchild,preschoolexchangetransfusion,wholebloodfemalehumaninfantlymphocytetransformationmalemonocytes/immunologyKWDplasmaexchangereticuloendotheliosis/blood/complications/KWDtherapysupport,uKWDsKWDgov't,pKWDhKWDsKWDjournalarticle
830130
M8310021

Copyright © 1983 - National Library of Medicine. Reproduced under license with the National Library of Medicine, Bethesda, MD.

AEGiS is a 501(c)3, not-for-profit, tax-exempt, educational corporation. AEGiS is made possible through unrestricted funding from Boehringer Ingelheim, Bridgestone/Firestone Charitable Trust, Bristol-Myers Squibb Company, Elton John AIDS Foundation, Gill Foundation, the National Library of Medicine, Quest Diagnostics, Roche and Trimeris, and donations from users like you. Always watch for outdated information. This article first appeared in 1983. This material is designed to support, not replace, the relationship that exists between you and your doctor.

AEGiS presents published material, reprinted with permission and neither endorses nor opposes any material. All information contained on this website, including information relating to health conditions, products, and treatments, is for informational purposes only. It is often presented in summary or aggregate form. It is not meant to be a substitute for the advice provided by your own physician or other medical professionals. Always discuss treatment options with a doctor who specializes in treating HIV.

Copyright ©1980, 1983. AEGiS. All materials appearing on AEGiS are protected by copyright as a collective work or compilation under U.S. copyright and other laws and are the property of AEGiS, or the party credited as the provider of the content. .