Hypergammaglobulinemia natural history
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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
- Viral
- Gastrointestinal tumors (carcinoid of pancreas, glucagonoma of pancreas)
- Lymphomas
- Epstein-Barr virus associated hodgkin's disease
- Infection
- 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)
- Liver disease
- 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
- ↑ 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.
- ↑ 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/
- ↑ 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.