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A familial case of hyper-IgM immunodeficiency.
Acta Haematol. 1992; 88(1):50-4.AH

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+Show Affiliations

First Department of Internal Medicine, Faculty of Medicine, Kyushu University, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

1414163

Citation

Iwakiri, R, et al. "A Familial Case of hyper-IgM Immunodeficiency." Acta Haematologica, vol. 88, no. 1, 1992, pp. 50-4.
Iwakiri R, Nakano T, Harada M, et al. A familial case of hyper-IgM immunodeficiency. Acta Haematol. 1992;88(1):50-4.
Iwakiri, R., Nakano, T., Harada, M., Nagafuchi, S., Teshima, T., Ono, N., Yamamoto, Y., & Niho, Y. (1992). A familial case of hyper-IgM immunodeficiency. Acta Haematologica, 88(1), 50-4.
Iwakiri R, et al. A Familial Case of hyper-IgM Immunodeficiency. Acta Haematol. 1992;88(1):50-4. PubMed PMID: 1414163.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A familial case of hyper-IgM immunodeficiency. AU - Iwakiri,R, AU - Nakano,T, AU - Harada,M, AU - Nagafuchi,S, AU - Teshima,T, AU - Ono,N, AU - Yamamoto,Y, AU - Niho,Y, PY - 1992/1/1/pubmed PY - 1992/1/1/medline PY - 1992/1/1/entrez SP - 50 EP - 4 JF - Acta haematologica JO - Acta Haematol. VL - 88 IS - 1 N2 - 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. SN - 0001-5792 UR - https://www.unboundmedicine.com/medline/citation/1414163/A_familial_case_of_hyper_IgM_immunodeficiency_ L2 - https://www.karger.com?DOI=10.1159/000204596 DB - PRIME DP - Unbound Medicine ER -