Immunodeficiency with hyperimmunoglobulin M
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
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| Immunodeficiency with hyperimmunoglobulin M | |
|---|---|
| |
| Synonyms | Hyper-IgM syndrome |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Recurrent infections, low levels of IgG, IgA, and IgE with normal or elevated IgM |
| Complications | Autoimmune disorders, liver disease, cancer |
| Onset | Usually in infancy or early childhood |
| Duration | Lifelong |
| Types | N/A |
| Causes | Genetic mutations affecting CD40 ligand or other related proteins |
| Risks | Male gender (for X-linked form) |
| Diagnosis | Blood tests showing immunoglobulin levels, genetic testing |
| Differential diagnosis | Common variable immunodeficiency, X-linked agammaglobulinemia |
| Prevention | N/A |
| Treatment | Immunoglobulin replacement therapy, antibiotics, hematopoietic stem cell transplantation |
| Medication | N/A |
| Prognosis | Variable, depending on severity and treatment |
| Frequency | Rare |
| Deaths | N/A |
Immunodeficiency with hyperimmunoglobulin M (also known as Hyper IgM syndrome) is a rare and complex primary immunodeficiency disease characterized by low levels of antibodies necessary for defending the body against bacterial or viral infections.
Symptoms[edit | edit source]
Patients with Hyper IgM syndrome may experience frequent and severe infections, including pneumonia, sinusitis, and skin infections. Other symptoms may include diarrhea, hepatitis, and encephalitis.
Causes[edit | edit source]
Hyper IgM syndrome is caused by mutations in several different genes, including the CD40 ligand gene, which is located on the X chromosome. This is why the disease is more common in males.
Diagnosis[edit | edit source]
Diagnosis of Hyper IgM syndrome is based on clinical symptoms, family history, and laboratory tests. These tests may include immunoglobulin levels, antibody response to vaccines, and genetic testing.
Treatment[edit | edit source]
Treatment for Hyper IgM syndrome is aimed at preventing and treating infections. This may include antibiotics, immunoglobulin replacement therapy, and in severe cases, hematopoietic stem cell transplantation.
See also[edit | edit source]
References[edit | edit source]
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Contributors: Prab R. Tumpati, MD
