RESUMO
Carriers for chronic granulomatous disease (CGD) and patients with lupus erythematosus (LE) share several characteristics: They are mostly females, they reduce nitroblue tetrazolium (NBT) poorly in their neutrophils, and, in some cases, they have similar skin lesions. We thus investigated 19 female LE patients for the presence of laboratory findings characteristic of the carrier state for CGD. None of these patients turned out to have the combined abnormality of neutrophil bactericidal activity and neutrophil NBT-reduction that is diagnostic of CGD carrier state in the X-linked form. An increased frequency of CGD carriers among female LE patients thus appear to be unlikely. Why some CGD carriers develop skin lesions typical of LE remains unexplained.
Assuntos
Doença Granulomatosa Crônica/genética , Disfunção de Fagócito Bactericida/genética , Cromossomos Sexuais , Adulto , Idoso , Atividade Bactericida do Sangue , Feminino , Doença Granulomatosa Crônica/diagnóstico , Humanos , Lúpus Eritematoso Sistêmico/genética , Pessoa de Meia-Idade , Neutrófilos/fisiologiaRESUMO
Neutrophil (PMN) bactericidal activity, phagocytosis, and nitroblue tetrazolium (NBT) reduction were evaluated in 18 children with untreated or relapsing acute leukemia and 20 children in hematologic remission. Half of the patients in relapse demonstrated abnormal PMN bactericidal activity, while remission patients had essentially normal PMN bactericidal activity. Phagocytosis of Staphylococcus aureus was normal in relapse and remission subjects. NBT reduction by PMN's of leukemic patients was significantly lower than that of controls, but there was no correlation between decreased NBT-reductase activity and decreased bactericidal power. Six patients in remission had received intensive chemotherapy for more than 4 years, and all demonstrated normal PMN functions. Among relapse patients with abnormal PMN bactericidal activity 63% eventually developed severe bacterial infections. By comparison, 20% of the relapse patients with normal PMN bactericidal activity subsequently developed severe infections. The PMN dysfunction observed in relapse patients suggests that abnormal PMN bactericidal activity may contribute the increased susceptibility to bacterial infections during leukemic relapse.
