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selective IgM deficiency

Epidemiology: - children & adults - no gender bias Pathlogy: - humoral immune dysfunction - innate immunity is relatively intact - T cell subsets, & T cell functions are normal Genetics: - 22q11.2 chromosome deletion in some cases Clinical manifestations: - infections with extracellular & intracellular bacteria, viruses, & fungi - allergies - increased prevalence of autoimmune disease Laboratory: - serum IgM - serum IgG, serum IgA. serum IgG subclasses for exclusion - Flow cytometry for B cells with surface IgM may be helpful Management: - patients with impaired pneumococcal antibody responses appear to respond to immune globulin [1] - fresh frozen plasma may be considered for severe infections Notes: - originally described in children with memingococcemia

General

humoral immune dysfunction

References

  1. Louis AG, Gupta S. Primary Selective IgM Deficiency: An Ignored Immunodeficiency. Clin Rev Allergy Immunol. 2013 Jun 12 PMID: 23760686
  2. Hussain H and Kaliner MA eMedicine: Immunoglobulin M Deficiency http://emedicine.medscape.com/article/137693-overview