Wiskott-Aldrich syndrome

Wiskott-Aldrich syndrome (WAS) is a X-linked, recessive immunodeficiency disease charcterised by thrombocytopenia, eczema and immunodeficeincy.

Cytogenetics

Wiskott-Aldrich syndrome is caused due to a mutation in the WASP gene. Thus the platelets are small in size and have a shortened half-life.

Immunological factors

IgM is decreased
IgE is increased
IgA and IgG normal
IgA initially is normal then high

Immune defects

These patients are unable to make antibodies to polysaccharide antigens normally. The response to protein antigens may also be impaired, late in the course of the disease. Also most patients may acquire T cell deficiencies and there is an increased risk of pneumococcal and herpes simplex infections.

Treatment

Transplantation of histocompatibile bone marrow from a matched donor is the popular method. In cases of patients lacking a suitable donor, intravenous immunoglobulin administartion can be given or splenectomy can be done.



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