Tödlicher Verlauf einer generalisierten BCG-Histiozytose bei angeborenem Immundefekt

: Severe combined X-linked immune deficiency ended fatally in a six-month-old male infant after generalized BCG infection. Damage in the cell-bound thymus-dependent immune system is decisive in the development of "BCG histiocytosis". As a result of absent immunocompetent sensitized T-lymphocytes there is insufficient information and stimulation of macrophages to kill the micro-organsims. The disorder of bactericidal properties of macrophages results in an overwhelming infection with BCG bacteria with a characteristic morphological picture.