Abstract
For the period 1994-1996, five boys with x-linked recessive form of chronic granulomatosis disease (CGD) were diagnosed and treated in Children's Oncohematology Clinic in Sofia, Bulgaria. The patients were hospitalized for anemia and marked hepatosplenomegaly. All 5 patients had BCG-lymphadenitis and some of them had fistulisation of the lymph nodes. The history of relapsing skin and lung infections, otitis and torpid evolution of a specific lymphadenitis raised the clinical suspicion of CGD. Primary immune deficiency in phagocytosis was documented with a positive NBT-test in all 5 patients, their mothers and some of their relatives.
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