A familial case of hyper-IgM immunodeficiency.

Abstract

A 22-year-old male was diagnosed as having immunodeficiency with hyper-IgM based upon recurrent pneumonia, marked elevation of serum IgM and markedly decreased level of IgG. IgG-or IgA-bearing B cells were not detected in peripheral blood while a number and a proportion of peripheral blood T lymphocytes were normal. Peripheral blood lymphocytes from this patient proliferated normally in response to T-independent and T-dependent B cell mitogens, and to T cell mitogens. Furthermore, the same type of dysgammaglobulinemia with increased IgM was found in the patient's father and brother. From these observations, it is suggested that it is a rare case of autosomal dominant or polygenal inheritance of hyper-IgM immunodeficiency.

• Authors: R Iwakiri, T Nakano, M Harada, S Nagafuchi, T Teshima, N Ono, Y Yamamoto, Y Niho
• Journal: Acta haematologica (Acta Haematol) Vol. 88 Issue 1 Pg. 50-4 ( 1992) ISSN: 0001-5792 [Print] Switzerland
• PMID: 1414163 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Immunoglobulin M

Topics

• Adult
• Biopsy
• CD4-CD8 Ratio
• Chronic Disease
• Dysgammaglobulinemia (genetics, immunology, pathology)
• Humans
• Immunoglobulin M (deficiency)
• Lymph Nodes (pathology)
• Male
• Pedigree
• Pneumonia (genetics, immunology, pathology)
• Recurrence