Our expert says:
ES, it's an X-linked recessive immunodeficiency disorder of male infants, characterized by eczema, thrombocytopenia, and recurrent infection.
The first manifestations often are hemorrhagic (usually bloody diarrhea), followed by recurrent respiratory infections. Malignancy (especially lymphoma and acute lymphoblastic leukemia) is common (10%) in survivors > 10 yr old. The characteristic immunologic defects include poor antibody responses to polysaccharide antigens, cutaneous anergy, partial T-cell immunodeficiency, elevated levels of IgE and IgA, low levels of IgM, and hypercatabolism of IgG but normal IgG levels. Because of the combined deficiency in both B- and T-cell function, infections occur with pyogenic bacteria, viruses, fungi, and P. carinii. Hematologically, these patients have small platelets and increased splenic destruction of platelets; accordingly, splenectomy may alleviate the thrombocytopenia. The gene defect has been localized to Xp11.
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