RESUMEN
Current concepts of pathogenesis and therapy of Wiskott-Aldrich syndrome are discussed, along with demonstrating the case history of a patient with the clinical features of this rare disorder. In addition to the known symptoms there was an elevated blood monocyte count together with a decreased total number of lymphocytes. Immunological analysis of the mononuclear cell fraction revealed an imbalance between mature T-cells, "natural-killer"-cells and monocytes.
Asunto(s)
Síndrome de Wiskott-Aldrich/diagnóstico , Preescolar , Humanos , Lactante , Recién Nacido , Células Asesinas Naturales , Linfopenia/diagnóstico , Masculino , Metilprednisolona/uso terapéutico , Monocitos , Linfocitos T , Síndrome de Wiskott-Aldrich/sangre , Síndrome de Wiskott-Aldrich/tratamiento farmacológicoRESUMEN
Since 1979 in a cooperative study (COALL-80) 110 children with acute lymphoblastic leukemia (ALL) and 13 children with high grade malignant non Hodgkin's lymphoma (NHL) have been treated with a less aggressive modification of the Westberlin Study Program BFM 76/79. Asparaginase has been delayed from the initial 4 drugs regimen and interposed in between induction therapy and treatment of central nervous system (CNS). Induction therapy that way was well tolerated and realized mostly on an outpatient basis. The expected 2 1/2 years disease free survival rate for ALL is 86% and no worse than the results of the original BFM study. We were able to define by age (greater than 2, less than 7 years), initial blast count (less than 25000/mm3) and immunological typing (cALL Ag+) a great group of patients (52%) which has remained in continuous remission as a whole. For this group further reduction of therapy intensity is planned (omission of cyclophosphamide during CNS-phase and use of intermediate dose methotrexate (MTX-mHD) instead of CNS-irradiation).
Asunto(s)
Leucemia Linfoide/terapia , Factores de Edad , Asparaginasa/administración & dosificación , Niño , Preescolar , Ensayos Clínicos como Asunto , Femenino , Alemania Occidental , Humanos , Leucemia Linfoide/mortalidad , Linfoma/terapia , Masculino , Estudios ProspectivosRESUMEN
Leucemic cells in 54 children with ALL have been typed immunologically by conventional antisera, monoclonal antibodies and receptor tests as c-ALL (62.5%), T-ALL/NHL (18.5%), B-ALL (1.9%) and AUL (16.6%), respectively. The different subtypes showed a noteworthy heterogeneity in receptor patterns which may reflect an arrest in discrete differentiation stages within the maturation pathway of a lymphoid cell population. The characteristic set of clinical symptoms of T-cell neoplasias could once again be confirmed. Of interest is a clear trend for a less favourable outcome in AUL as compared to c-ALL and in NHL versus ALL as a whole, whereas no difference could be found between T-ALL and non T-ALL during therapy. Immunological typing of ALL is of clinical importance as entities with different biological behaviour and, correspondingly, different therapeutical requirements can be distinguished.