RESUMO
Successful treatment of a patient with myelokathexis, a rare form of chronic neutropenia associated with recurrent infections, is described. Rapid mobilization of bone marrow neutrophils and improved myeloid morphologic features were observed after treatment with human granulocyte colony stimulating factor. Transient thrombocytopenia and bone pain were observed during treatment. Although neutrophil chemotaxis, superoxide production, and FcRIII surface expression were reduced, the patient improved clinically after restoration of a normal neutrophil count.
Assuntos
Fator Estimulador de Colônias de Granulócitos/uso terapêutico , Neutropenia/terapia , Antígenos CD/análise , Quimiotaxia de Leucócito , Criança , Doença Crônica , Feminino , Gengivite/imunologia , Gengivite/terapia , Fator Estimulador de Colônias de Granulócitos/efeitos adversos , Humanos , Neutropenia/imunologia , Neutrófilos/metabolismo , Neutrófilos/fisiologia , Contagem de Plaquetas , Proteínas Recombinantes/efeitos adversos , Proteínas Recombinantes/uso terapêutico , Estomatite/imunologia , Estomatite/terapiaRESUMO
Chronic benign neutropenia of childhood is a heterogeneous disorder. This study describes two severely neutropenic children with a benign clinical course and the unique bone marrow finding of macrophage engulfment of band and segmented neutrophils. Phagocytic vacuoles in the majority of macrophages contained neutrophils in various stages of digestion at both the light and electron microscope level (with as many as four neutrophils observed in single macrophages). Ultrastructural studies demonstrated that the neutrophils were morphologically normal, and apparently viable at the time of phagocytosis. This type of neutrophil phagocytosis was not observed in five other consecutively studied children with CBN. All of these children with CBN had ultrastructurally normal neutrophils, lacked demonstrable antineutrophil antibodies or serum inhibitors to in vitro myelopoiesis, and had normal colony-forming cells and colony-stimulating activity in vitro. Thus despite many similar clinical and laboratory features in children with CBN, only a unique subgroup of these patients demonstrates abnormal neutrophil-macrophage interaction.
Assuntos
Agranulocitose/imunologia , Medula Óssea/patologia , Macrófagos/imunologia , Neutropenia/imunologia , Fagocitose , Adolescente , Adulto , Células Cultivadas , Criança , Pré-Escolar , Doença Crônica , Feminino , Humanos , Macrófagos/ultraestrutura , Masculino , Neutropenia/patologia , Neutrófilos/imunologia , Neutrófilos/ultraestruturaRESUMO
The records of 22 children with the diagnosis of idiopathic immune hemolytic anemia were reviewed. Fifty per cent of the children had a history of an antecedent infection and 32% had underlying immune deficiency or lymphoproliferative diseases. The majority (77%) of the patients had an acute self-limited disease which was frequently associated with a positive complement or nongamma Coombs test. The anemia was usually severe, and a number of the children had reticulocytopenia at the time of diagnosis. These children responded rapidly to transfusions and corticosteroids and generally remained well on follow-up. Immunosuppressive drugs did not prove useful in two patients refractory to prednisone, but splenectomy was apparently beneficial in three of four patients so treated. Mortality rate was 18%, with most of the deaths related to an underlying disease.