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
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