RESUMEN
Hepatic veno-occlusive disease (VOD) of the liver is a common complication following high-dose cytotoxic therapy for bone marrow transplantation (BMT). Liver injury is believed to occur following free radical damage to endothelial cells of the sinusoids and small hepatic veins. Glutathione the main antioxidant of the cytosol becomes depleted following chemotherapy. Animal studies have shown that glutamine infusions can maintain glutathione levels and protect against free radical injury. We present two cases of established VOD successfully treated with intravenous glutamine (as dipeptide) and oral vitamin E. Although both cases have possible confounding factors we believe that these give support to the notion that glutamine/vitamin E may have a role in the prophylaxis and treatment of VOD. Further formal trials are indicated.
Asunto(s)
Antineoplásicos/efectos adversos , Glutamina/administración & dosificación , Enfermedad Veno-Oclusiva Hepática/tratamiento farmacológico , Vitamina E/administración & dosificación , Adulto , Humanos , MasculinoRESUMEN
Pancytopenia with severe bone marrow dysplasia following allogeneic bone marrow transplantation for acute myeloid leukemia (M6) may pose a diagnostic problem. We report a case of M6 acute myeloid leukemia in which progressive macrocytosis, pancytopenia and severe bone marrow dysplasia induced by acetazolamide therapy developed after successful engraftment of a donor marrow. We discuss the diagnostic problems and the usefulness of conventional cytogenetics and interphase fluorescence in situ hybridisation in excluding recipient myelodysplasia relapse. We also suggest that acetazolamide should be used with caution, especially following bone marrow transplantation.
Asunto(s)
Acetazolamida/efectos adversos , Trasplante de Médula Ósea , Síndromes Mielodisplásicos/diagnóstico , Pancitopenia/inducido químicamente , Adolescente , Femenino , Humanos , Leucemia Eritroblástica Aguda/complicaciones , Leucemia Eritroblástica Aguda/terapia , Osteogénesis Imperfecta/complicaciones , Pancitopenia/diagnóstico , Trasplante HomólogoRESUMEN
Autologous GVHD was induced using CsA and alpha-IFN in a patient undergoing autologous PBSCT for accelerated phase CML. We demonstrated that the autologous mixed lymphocyte reactions were extremely sensitive and specific for the detection of the GVHD when compared to skin biopsy. The resultant autologous GVHD was associated with an in vitro GVL effect, suggesting a potential clinical benefit of this therapeutic manoeuvre. The post-PBSCT period was associated with an improvement in normal haemopoiesis and reduction in the proportion of blood cells expressing the Philadelphia chromosome.
Asunto(s)
Antineoplásicos/administración & dosificación , Ciclosporina/administración & dosificación , Enfermedad Injerto contra Huésped/inducido químicamente , Trasplante de Células Madre Hematopoyéticas , Inmunosupresores/administración & dosificación , Interferón-alfa/administración & dosificación , Leucemia Mielógena Crónica BCR-ABL Positiva/terapia , Humanos , Masculino , Persona de Mediana Edad , Trasplante AutólogoRESUMEN
Dominant transmission of a variant of von Willebrand's disease (vWD) with aberrant polymerization of von Willebrand factor (vWF) has been identified in a Scottish family. Multimer analysis of plasma vWF from the propositus and her father revealed an identical pattern to that previously reported in families designated as type IID vWD. There is loss of the larger multimers and presence of an intermediate subsidiary band not seen in normal subjects or other vWD variants. Platelet/vWF interaction induced by ristocetin is not enhanced in these cases and the platelet vWF shows the same aberrant multimer pattern as plasma vWF. DDAVP infusion in two affected members of the Scottish family and in one of the index cases produced a rise in plasma vWF antigen and factor VIII. Higher molecular weight vWF multimers appeared transiently after infusion of desmopressin (1-deamino-8-D-arginine vasopressin, abbreviated DDAVP) coincident with shortening of the bleeding time. The platelet counts did not change after the DDAVP infusions. DDAVP should be considered for management of bleeding in this variant of von Willebrand's disease.
Asunto(s)
Desamino Arginina Vasopresina/uso terapéutico , Enfermedades de von Willebrand/tratamiento farmacológico , Factor de von Willebrand/análisis , Adulto , Plaquetas/análisis , Femenino , Humanos , Sustancias Macromoleculares , Masculino , Enfermedades de von Willebrand/sangreAsunto(s)
Alantoína , Compuestos de Cetrimonio , Hexaclorofeno , Miristatos , Ácidos Mirísticos , Ácidos Nicotínicos , Úlcera por Presión/prevención & control , Compuestos de Amonio Cuaternario , Siliconas , Simeticona , Escualeno , Ácidos Esteáricos , Administración Tópica , Anciano , Anciano de 80 o más Años , Ensayos Clínicos como Asunto , Método Doble Ciego , Combinación de Medicamentos , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Clinical and laboratory features at presentation were correlated with morphological (FAB) subclass of AML in a group of 112 children diagnosed between 1972 and 1982. Patients with a monocytic component of AML (M4, M5) had higher initial leucocyte counts, a higher incidence of extramedullary infiltration and of CNS involvement. In M4 AML CNS relapse occurred in patients with a high initial leucocyte count whereas in M5 AML CNS involvement tended to occur at presentation in children with low initial counts. Two-thirds of patients treated achieved remission and most failures were due to inadequate chemotherapy, although haemorrhage, leucostasis or metabolic complications caused early death in patients with M4 and M5 AML. With a minimum follow up of 3 years only 12% of patients are alive; these figures have not improved in consecutive series despite increasing intensity of induction and more recent availability of bone marrow transplantation. No features predictive of long-term survival were identified, but patients with myeloid differentiation (M1, M2, M3) did better than those with a monocytic or erythroid component (M4, M5, M6). The proportion of patients with AML curable by chemotherapy seems unlikely to increase without marked intensification of post-remission chemotherapy. More aggressive CNS prophylaxis may be of benefit in cases with a monocytic component.
Asunto(s)
Leucemia Mieloide Aguda/patología , Neoplasias Encefálicas/secundario , Niño , Preescolar , Femenino , Humanos , Fragmentos Fab de Inmunoglobulinas/análisis , Lactante , Leucemia Mieloide Aguda/sangre , Leucemia Mieloide Aguda/mortalidad , Leucemia Mieloide Aguda/terapia , Recuento de Leucocitos , Masculino , Pronóstico , Neoplasias de la Médula Espinal/secundarioRESUMEN
Case reports of four girls and one boy with acute lymphoblastic leukaemia (ALL) or acute myeloid leukaemia (AML) and t(4;11) are presented. The incidence of t(4;11) ascertained at diagnosis in ALL was 2.6% and in AML 5.3%. Four of the children were under 2 years and one was 11 years at diagnosis. Leucocyte counts above 71 X 10(9)/l and liver, spleen and node enlargement were found in all cases. Blasts of the four cases tested at diagnosis were negative to the c-ALL antigen and either TdT+ (ALL) or TdT- (AML M1). Maximum survival was less than 8 months. Additional chromosomal change was found at diagnosis in two cases and in relapse in a third. In the case of AML t(4;11) (q21;p15) was present as a second translocation. Additional numerical changes, in these and other reported cases, included + 6, commonly found in ALL, +8, +19, more often reported in AML. It is suggested that additional chromosomal changes in these cases support cytochemical and surface marker evidence that t(4;11) has a pluripotent target cell, similar to that of the Philadelphia translocation.
Asunto(s)
Cromosomas Humanos 4-5 , Cromosomas Humanos 6-12 y X , Leucemia Linfoide/genética , Leucemia Mieloide Aguda/genética , Translocación Genética , Adolescente , Aberraciones Cromosómicas , Femenino , Humanos , Lactante , Cariotipificación , MasculinoRESUMEN
Fifty-four unrelated children with steroid responsive nephrotic syndrome of childhood were studied for 24 alleles at the HLA--A and B loci. A significantly increased incidence of HLA--B8 (Pc less than 0.01) was observed compared to controls. No association between response to cyclophosphamide therapy and HLA antigens was seen.
Asunto(s)
Corticoesteroides/uso terapéutico , Antígenos HLA/genética , Síndrome Nefrótico/inmunología , Niño , Preescolar , Ciclofosfamida/uso terapéutico , Haploidia , Humanos , Síndrome Nefrótico/tratamiento farmacológico , Síndrome Nefrótico/etiologíaRESUMEN
Sixty-seven consecutive patients with acute myeloid leukaemia (AML), who presented to the unit over a 5-year period, were reviewed. Remission rates, length of remission and duration of survival were studied. Twenty-seven patients (40.3%) achieved complete remission (CR). Eleven patients (16.4%) achieved a second CR. The median survival of patients who achieved a CR was 15 months. Median survival of patients who failed to go into remission was one month. Median duration of the first remission was 7 months. Three patients are still alive and in remission. These results are discussed in relation to those reported from other centres.