MHC class 2 deficiency and X-linked agammaglobulinaemia in a consanguineous extended family

Int J Immunogenet. 2009 Aug;36(4):223-6. doi: 10.1111/j.1744-313X.2009.00847.x. Epub 2009 May 19.

Abstract

Manifestations of immunodeficiency within the same family are presumed to be the same disease. We report a consanguineous extended family where four patients have immunodeficiency, three have X-linked agammaglobulinaemia and one has major histocompatibility complex class 2 deficiency. Within one family, two rare genetic diseases with similar clinical manifestations can occur.

Publication types

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

MeSH terms

  • Agammaglobulinaemia Tyrosine Kinase
  • Agammaglobulinemia / drug therapy
  • Agammaglobulinemia / genetics*
  • Agammaglobulinemia / immunology
  • Consanguinity
  • Exons / genetics
  • Exons / immunology
  • Female
  • Genetic Diseases, X-Linked / drug therapy
  • Genetic Diseases, X-Linked / genetics*
  • Genetic Diseases, X-Linked / immunology
  • Histocompatibility Antigens Class II / genetics*
  • Histocompatibility Antigens Class II / immunology
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Infant
  • Introns / genetics
  • Introns / immunology
  • Male
  • Pedigree
  • Promoter Regions, Genetic
  • Protein-Tyrosine Kinases / genetics*
  • Protein-Tyrosine Kinases / immunology

Substances

  • Histocompatibility Antigens Class II
  • Immunoglobulins, Intravenous
  • Protein-Tyrosine Kinases
  • Agammaglobulinaemia Tyrosine Kinase