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Immunoglobulin M Deficiency

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Acute

What is Immunoglobulin M Deficiency?

Selective immunoglobulin M deficiency (sIgMD) is a rare immune disorder that has been reported in association with serious infections, such as bacteremia. The disorder can occur in infants, children, and adults.

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Individuals with selective IgM deficiency may be asymptomatic or symptomatic. Of those who are symptomatic, approximately 80% present with predominant bacterial infections. Among infections, the most common are chronic sinusitis, upper respiratory tract infections, bronchitis, and pneumonia.

Individuals with selective IgM deficiency may be asymptomatic or symptomatic. Of those who are symptomatic, approximately 80% present with predominant bacterial infections. Among infections, the most common are chronic sinusitis, upper respiratory tract infections, bronchitis, and pneumonia

The pathogenesis of SIgMD is unclear, although there are likely multiple mechanisms that can result in isolated low serum IgM, such as decreased T helper cell activity, increased isotype-specific regulatory T cells, increased CD8 + T cells, intrinsic B cell defect, increased regulatory B cells, defective secretion of μ ...

Other causes of decreased levels of serum IgM (i.e., secondary IgM deficiency) are episodes of infection, thymic hypoplasia, celiac disease, autoimmune disease, and certain adult malignancies; and other PIDs (Wiskott-Aldrich syndrome, DOCK8 deficiency, ataxia-telangiectasia, CVID, and XLA, in combination with IgG and ...

There is no specific treatment to correct low IgM levels; however, individuals who have this condition and also have impaired responses to vaccine antigens, particularly pneumococcal polysaccharide vaccines, may require immunoglobulin (Ig) replacement therapy.

How is it diagnosed?

How is it diagnosed?

Management. Selective IgM patients are managed in the same fashion as other antibody deficiencies. IRT is given if there is a significant associated functional antibody deficiency. Prophylactic antibiotics and prompt treatment of febrile illness are crucial.

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How is it treated?

Treatment for acute myeloid leukemia is vital. It varies with the patient and stage of the disease. Treatment options include

For such patients, intravenous immunoglobulin (IVIG) replacement may be an option. Prophylactic and/or rigorous therapeutic antibiotic treatments may be beneficial. Fresh frozen plasma may be considered for severe infections.

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