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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).
-
-