Familial combined cellular and humoral immune defect with multisystem granulomata.
نویسندگان
چکیده
A brother and sister presented with recurrent respiratory infections. Investigations showed combined cellular and humoral immune defects and non-caseating granulomata in lungs, liver, lymph nodes, and skin. Despite antimycobacterial chemotherapy and corticosteroids in the brother and the additional treatment of human immunoglobulin injections in the sister both patients deteriorated with pulmonary and hepatic involvement.
منابع مشابه
Sarcoidosis associated with combined immunodeficiency.
The association of multiple non-caseating granulomata and a positive Kviem test is normally considered to be indicative of a diagnosis of sarcoidosis. However, although depressed cell-mediated immunity is commonly described, it is extremely rare to find a humoral immune paresis. A patient is reported who had multiple granulomata, depressed cellular and humoral immunity and a positive Kveim test.
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عنوان ژورنال:
- Thorax
دوره 33 1 شماره
صفحات -
تاریخ انتشار 1978