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Introduction: Hyper immunoglobulin-M syndrome (HIGM) is a rare, x-linked recessive inherited disorder with Increased or normal serum IgM and decreased IgA, IgG and IgE and normal peripheral B cells. In this disease, we have defect in class switch recombination (CSR) and somatic hyper mutation that is caused by defects including the enzymes required for CSR and SHM. (SHM) Methods: To update about HIGM, PubMed and Google Scholar database were searched for term, X linked Hyper IgM syndrome and CD40 ligand. In our first search, we received about 90 updated articles, and then we distributed these articles among the authors. After reading the articles and sharing together, finally 33 articles were selected that fully related to the topic as references. Findings: In conclusion, HIGM is rare disease that starts early in life with lifelong recurrent attacks of various infections by a variety of symptoms, including lymph Adenopathy, vomiting, diarrhea, skin lesion and abdominal pain. Allogenic hematopoietic cell transplantation and BMT can be effective in these patients. Conclusion: In conclusion, HIGM is rare disease that starts early in life with lifelong recurrent attacks of various Infections by a variety of symptoms, including lymphadenopathy, vomiting, diarrhea, skin lesions and abdominal pain. Allogenic hematopoietic cell transplantation and BMT can be effective in these patients.

     

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