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1996-01-30
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Document 0430
DOCN M9610430
TI Immunodeficiency with hyperimmunoglobulinemia M in two female patients
is not associated with abnormalities of CD40 or CD40 ligand expression.
DT 9601
AU Oliva A; Quinti I; Scala E; Fanales-Belasio E; Rainaldi L; Pierdominici
M; Giovannetti A; Paganelli R; Aiuti F; Pandolfi F; Department of
Allergy and Clinical Immunology, La Sapienza,; University of Rome,
Italy.
SO J Allergy Clin Immunol. 1995 Sep;96(3):403-10. Unique Identifier :
AIDSLINE MED/96013056
AB The immunologic defect in X-linked immunodeficiency and
hyperimmunoglobulinemia M (HIM) are related to defective expression of
the CD40 ligand (CD40L). We have studied two female patients with HIM to
evaluate the role of CD40/CD40L in the pathogenesis of impaired
immunoglobulin switching. In addition to recurrent infections
characteristic of humoral immunodeficiencies, the two patients had
chronic hepatitis caused by type C virus. Phenotypic characterization of
peripheral blood mononuclear cells showed a similar picture in both
patients, with a reduction in the absolute numbers of CD4 cells and
increased numbers of CD8 and CD3/DR cells. B cells (CD19+) were reduced
in one patient, but CD40 was expressed on all CD19+ cells in both
patients. The expression of CD40L was normal on peripheral blood
mononuclear cells from the two patients with HIM on both resting and
stimulated cells. The combination of anti-CD40 and cytokines
(interleukin-2, interleukin-4, and interleukin-10) was able to restore
proliferative capacity to anti-IgM. Peripheral blood mononuclear cells
from the two patients with HIM showed a high spontaneous production of
IgM in vitro and no production of IgG or IgE. Our data suggest that the
defect of isotype switching in female patients with HIM is not related
to defective expression of the CD40/CD40L receptor system. A possible
role for chronic hepatitis C virus infection in the pathogenesis of the
disease is suggested by the detection of specific production of
anti-hepatitis C virus IgM.
DE Adolescence Aged Antigens, CD40/*METABOLISM Case Report Cell
Division Female Hepatitis C/COMPLICATIONS Human IgM/*METABOLISM
Immunoglobulins/METABOLISM Immunologic Deficiency
Syndromes/*BLOOD/COMPLICATIONS Ligands Membrane
Glycoproteins/*METABOLISM Monocytes/PATHOLOGY Support, Non-U.S. Gov't
JOURNAL ARTICLE
SOURCE: National Library of Medicine. NOTICE: This material may be
protected by Copyright Law (Title 17, U.S.Code).