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****Disseminated
****Disseminated
****Opportunistic Pneumocystis jirovecii pneumonia infections
****Opportunistic Pneumocystis jirovecii pneumonia infections
**Gastrointestinal tumors
**Gastrointestinal tumors (carcinoid of pancreas, glucagonoma of pancreas)
*The activation-induced cysteine deaminase (AICDA) deficient patients are at an increased risk of developing inflammatory as well as autoimmune diseases like autoimmune hemolytic anemia and autoimmune thrombocytopenia.<ref name="pmid14962793">{{cite journal| author=Quartier P, Bustamante J, Sanal O, Plebani A, Debré M, Deville A et al.| title=Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency. | journal=Clin Immunol | year= 2004 | volume= 110 | issue= 1 | pages= 22-9 | pmid=14962793 | doi=10.1016/j.clim.2003.10.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14962793  }} </ref>
**Lymphomas
***Epstein-Barr virus associated hodgkin's disease
*Life-threatening complications in adolescents and young adults with x-linked hyper-Ig M include:<ref>Johnson J, Filipovich AH, Zhang K. X-Linked Hyper IgM Syndrome. 2007 May 31 [Updated 2013 Jan 24]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2016. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1402/</ref>
**Liver disease
***Primary cirrhosis
***Carcinomas (bile duct carcinoma, hepatocellular carcinoma, adenocarcinomas of the liver and gall bladder)
*The activation-induced cysteine deaminase (AICDA) deficient patients are at an increased risk of developing inflammatory as well as autoimmune diseases like chronic arthritis, autoimmune hemolytic anemia, and autoimmune thrombocytopenia.<ref name="pmid14962793">{{cite journal| author=Quartier P, Bustamante J, Sanal O, Plebani A, Debré M, Deville A et al.| title=Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency. | journal=Clin Immunol | year= 2004 | volume= 110 | issue= 1 | pages= 22-9 | pmid=14962793 | doi=10.1016/j.clim.2003.10.007 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14962793  }} </ref>


==Prognosis==
==Prognosis==

Latest revision as of 16:47, 2 March 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]

Overview

Natural History

Complications

  • Common complications of x-linked hyper-Ig M include:[1]
    • Infection
      • Viral
        • Disseminated cytomegalovirus and herpes simplex virus infections
      • Bacterial
        • Cholangitis from Cryptosporidium parvum
      • Fungal
        • Disseminated
        • Opportunistic Pneumocystis jirovecii pneumonia infections
    • Gastrointestinal tumors (carcinoid of pancreas, glucagonoma of pancreas)
    • Lymphomas
      • Epstein-Barr virus associated hodgkin's disease
  • Life-threatening complications in adolescents and young adults with x-linked hyper-Ig M include:[2]
    • Liver disease
      • Primary cirrhosis
      • Carcinomas (bile duct carcinoma, hepatocellular carcinoma, adenocarcinomas of the liver and gall bladder)
  • The activation-induced cysteine deaminase (AICDA) deficient patients are at an increased risk of developing inflammatory as well as autoimmune diseases like chronic arthritis, autoimmune hemolytic anemia, and autoimmune thrombocytopenia.[3]

Prognosis

References

  1. Fried AJ, Bonilla FA (2009). "Pathogenesis, diagnosis, and management of primary antibody deficiencies and infections". Clin Microbiol Rev. 22 (3): 396–414. doi:10.1128/CMR.00001-09. PMC 2708392. PMID 19597006.
  2. Johnson J, Filipovich AH, Zhang K. X-Linked Hyper IgM Syndrome. 2007 May 31 [Updated 2013 Jan 24]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2016. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1402/
  3. Quartier P, Bustamante J, Sanal O, Plebani A, Debré M, Deville A; et al. (2004). "Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency". Clin Immunol. 110 (1): 22–9. doi:10.1016/j.clim.2003.10.007. PMID 14962793.

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