RESUMO
A patient with multinodular haemangiomatosis of the liver, rapidly deteriorating in the first weeks of life due to severe progressive congestive heart failure, was successfully treated by hepatic artery catheter embolisation at the age of 6 weeks.
Assuntos
Embolização Terapêutica , Hemangioma/terapia , Artéria Hepática , Neoplasias Hepáticas/terapia , Feminino , Humanos , Lactente , Regressão Neoplásica Espontânea , Neoplasias CutâneasRESUMO
This report deals with the case history of a 21/2-year old Turkish boy, who died from an overwhelming mononucleosis infectiosa. The parents, first cousins, lost already two sons and a daughter. According to the parents, the symptoms of these children resembled those of this boy. In the patient, described here, an acquired immunodeficiency was found, probably induced by the Epstein-Barr virus on the basis of a genetic susceptibility for this virus. The family history, the clinical picture, together with the serological, immunological - and histological examinations, pointed to the diagnosis of X-linked recessive lymfoproliferative syndrome (XLP), as described by Purtilo. The immunological findings resemble those of AIDS (acquired immunodeficiency syndrome).
Assuntos
Mononucleose Infecciosa/complicações , Transtornos Linfoproliferativos/genética , Cromossomo X , Síndrome da Imunodeficiência Adquirida/complicações , Pré-Escolar , Consanguinidade , Feminino , Genes Recessivos , Ligação Genética , Humanos , Transtornos Linfoproliferativos/complicações , Masculino , LinhagemRESUMO
The proliferating skin cells in a case of acute disseminated histiocytosis-X (Abt-Letterer-Siwe disease) confirmed by electron microscopy, were characterized by a panel of monoclonal antibodies using an immunoperoxidase technique. The "histiocytes" were found to stain with OKT-6 (anti-T6) and anti-HLA-DR antibodies. Unexpectedly, slight staining was also observed with Leu 3a (anti-T4) and OKM-1. A proliferative process of T4, T6, HLA-DR, OKM-1 positive Langerhans' cells has not yet been described and may be specific for histiocytosis-X.