Assuntos
Histiocitose de Células de Langerhans/complicações , Doenças da Glândula Tireoide/complicações , Obstrução das Vias Respiratórias/complicações , Obstrução das Vias Respiratórias/cirurgia , Diabetes Insípido/complicações , Seguimentos , Histiocitose de Células de Langerhans/fisiopatologia , Humanos , Hipotireoidismo/complicações , Lactente , MasculinoAssuntos
Histiocitose de Células de Langerhans/imunologia , Hipergamaglobulinemia/etiologia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Hemaglutininas/análise , Histiocitose de Células de Langerhans/complicações , Humanos , Imunoglobulina M/análise , Imunoglobulinas/análise , Lactente , Recém-Nascido , Ativação LinfocitáriaRESUMO
Histiocytosis X is characterized by the presence of cytoplasmic rod structures called Langerhans' cell granules or X granules (XG). It has been speculated that histiocytosis X is a Langerhans' cell disorder. This ultrastructural study was performed to quantitate the number of XG containing histiocytes in the histiocytosis X lesions. Twenty-four specimens from 22 patients with histiocytosis X were studied: 4 from skin, 5 from lymph node, 11 from bone, 2 from lung, 1 from gingiva, and 1 from cheek. The majority of the histiocytes in histiocytosis X lesions do not contain X granules. The majority of the histiocytes in histiocytosis X lesions do not contain X granules. The percentage of histiocytes with XG in a lesion has no relation to the age of the patient or the organ from which it was obtained, except for skin, where they were quite numerous. The relative percent-age of histiocytes with granules does not correlate significantly with the prognosis of these patients.
Assuntos
Grânulos Citoplasmáticos/ultraestrutura , Histiocitose de Células de Langerhans/patologia , Adolescente , Biópsia , Criança , Pré-Escolar , Seguimentos , Histiócitos/ultraestrutura , Humanos , Lactente , Recém-Nascido , PrognósticoRESUMO
It is now clear that the prognosis in children with histiocytosis X has improved considerable over the past few years. To be sure, patients with solitary lesions have an excellent prognosis. Whereas the outlook for patients with significant visceral involvement is not as good as those with bone lesions only, the outlook is by no means hopeless, as was once thought. A number of prognostic factors have been reviewed here. The most significant of these factors at the present time would appear to be age of onset of the disease, extent of involvement, the rapidity of progression of the disease, and, in particular, the presence or absence of dysfunction of such crucial organ systems as liver, lung, and hemopoietic system. Further studies of the significance of histologic features and immunologic findings are clearly needed to further our understanding of this disorder.
Assuntos
Histiocitose de Células de Langerhans , Fatores Etários , Pré-Escolar , Histiocitose de Células de Langerhans/imunologia , Histiocitose de Células de Langerhans/patologia , Histiocitose de Células de Langerhans/terapia , Humanos , Lactente , PrognósticoRESUMO
A prospective study for histiocytosis X was designed to determine whether "good risk" patients, ie, those without evidence of dysfunction of liver, lung, or hemopoietic system, would respond to single agent therapy; in this case chlorambucil (CMB) used in a dose of 5 mgm/m2/day. If there was no response after an adequate trial period, treatment was initiated with four drugs using a combination of prednisone, vinblastine, cyclophosphamide and methotrexate. There were 26 evaluable patients, 57% of whom were less than two years of age at onset of therapy. There were three complete and four partial responses to CMB for a response rate of 26.9%. Sixteen patients received an adequate trial of four-drug therapy with three complete and two partial responses for a response rate of 33%. These responses were inferior to those previously reported for either single agents or combined therapy in histiocytosis X.
Assuntos
Clorambucila/uso terapêutico , Histiocitose de Células de Langerhans/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Ensaios Clínicos como Assunto , Feminino , Humanos , Lactente , Masculino , Estudos Prospectivos , RiscoRESUMO
The three treatment regimens evaluated in this study (vinblastine alone versus prednisone and vinblastine versus prednisone and 6-mercaptopurine) proved to be about equally efficacious in children with histiocytosis x. Inasmuch as many physicians regard this disorder as one with a high mortality rate, it is worth emphasizing that 59 of the 83 patients (71% are living).
