Immunological aspects of immunodeficiency diseases part 79

Immune 3

 

COMPLEX IMMUNODEFICIENCIES DUE TO MISCELLANEOUS DEFECTS

WISCOTT-ALDRICH SYNDROME

Patients with Wiskott-Aldrich syndrome typically develop eczema; purpura due to thrombocytopenia  with small-sized, defective platelets; and a variable immunodeficiency. Antibody production to bacterial capsular polysaccharides is deficient. Patients therefore commonly develop recurrent sinopulmonary infections. T-cell and NK-cell function is deficient and progressive T lymphopenia develops with time. Hence, patients can develop opportunistic infections. Risk of malignancy (especially leukimias or EBV-induced lymphomas) is increased in these patients.

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