Hyper_Igm_Syndrome
Hyper_Igm_Syndrome Causes
An inherited disorder, Hyper IgM syndrome triggers a number of immunodeficiencies, or a state in which the immune system becomes too weak to fight infections. Hyper IgM syndrome is typically marked by low levels of neutrophil cells, which help fight infections in the body. As a result, individuals suffering from Hyper IgM syndrome are more prone to certain infectious diseases. Immunoglobulin M, or IgM, is basically an antibody produced by all healthy B cells. When exposed to a recognized antigen, B cells transform to a new class of antibodies to ward off viruses, bacteria, and other pathogens. For those with hyper IgM syndromes, their B cells are unable to switch to a different type of antibody, thus producing abnormally high IgM antibodies instead. In effect, their B Cells overproduce IgM and, consequently, underproduce all other kinds of antibodies. Variations of hyper-IgM syndromes include deficiencies in other immunoglobulin classes, such as IgA, IgG, and IgE.
Hyper_Igm_Syndrome Definition
Hyper IgM syndrome, or ?Hypogammaglobulinemia with Hyper-IgM', is a type of genetic disease characterized by a distinctly high occurrence of Immunoglobulin M (i.e., IgM) antibodies.
Hyper_Igm_Syndrome Diagnosis
Hyper IgM syndrome is diagnosed in individuals with extremely high levels of IgM. More commonly, however, the disorder may also be found on people with otherwise normal IgM levels but abnormally low levels of IgG and IgA (the two other major types of antibodies). A clinical test is now available which can reliably confirm or disprove the diagnosis. It is now also possible to assess DNA from female relatives to see if they are carriers of this particular genetic abnormality. A prenatal diagnostic test may also be done very early in the pregnancy.
Hyper_Igm_Syndrome Symptoms and Signs
The most frequent symptoms are intermittent infections, including ear, throat, and chest infections. If the immunodeficiency is not diagnosed or treated, permanent damage to ears and lungs may occur. Hyper IgM syndrome patients are susceptible to chronic pneumonia ? in particular, PCP (or Pneumocystis carinii pneumonia), which often occurs in T cell immunodeficiencies. Additionally, other infectious problems may result ? including diarrhea, bones and joints disorders (such as osteomyelitis, or bone infection), mouth ulcers, anemia, and even liver problems, among others.
Hyper_Igm_Syndrome Treatment
Until very recently, Hyper IgM syndrome patients have been commonly treated with immunoglobulin replacement therapy. Most patients are still undergoing this lifelong treatment. However, due to the high turnout of malignant liver diseases, bone marrow transplantation (or BMT) is now a more acceptable form of treatment for affected individuals. Illegible candidates for BMT can be successfully cured of Hyper IgM syndrome.