Hyper IgM Syndromes
What is it?
IgM is immunoglobulin M, which is one type of antibody. In hyper-IgM syndromes, some of the other immunoglobulins are turned into IgM antibodies, which causes decreased levels of the other immunoglobulins. Therefore, people who suffer from these syndromes are more susceptible to infections and sometimes even malignancies. The most common form of hyper IgM syndrome is caused due to a lack of protein found on the surface of T-lymphocytes, which have a crucial role in the immune system. Hyper IgM syndrome is inherited in either an X-linked pattern or autosomal recessive.
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Hyper IgM Syndromes.
Additional names
This group contains additional names:
- X-linked severe combined immunodeficiency
Signs & symptoms
Symptoms usually develop during the first year or two of life. The most common problem is recurrent infections, typically upper and lower respiratory tract bacterial infection. In some of the cases, fungal infections are also possible. Gastrointestinal symptoms such as diarrhea, liver disease and malabsorption. Neutropenia, or low white blood cell count, is seen in many of the patients. Autoimmune disorders such as arthritis, hemolytic anemia and hypothyroidism may also occur.
Diagnosis
Diagnosis of hyper IgM syndromes should be suspected in babies with recurrent infections. The exact diagnosis is done by DNA analysis.
Treatment
Treatment includes immunoglobulin replacement therapy, as in all forms of the disease there is a lack in IgG. Also, prophylactic treatment of antibiotics is sometimes recommended due to their susceptibility to PCP and other infections.
☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.
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