RESUMO
A significant proportion of patients relapse after allogeneic BMT for CML. These relapses have been treated by induction of a graft-versus-leukemia effect by transfusing donor leukocytes. We have treated a 27-year-old woman with interferon and donor leukocyte transfusion and a complete haematological and cytogenetic remission was obtained coincident with the onset of GVHD. Her course was complicated by prolonged and profound pancytopenia which was fully reversed by the administration of rGM-CSF. She remains in CR with mild dermatomyositis due to chronic GVHD 17 months after the procedure.
Assuntos
Transplante de Medula Óssea , Dermatomiosite/etiologia , Doença Enxerto-Hospedeiro/complicações , Fatores Imunológicos/uso terapêutico , Interferon-alfa/uso terapêutico , Leucemia Mielogênica Crônica BCR-ABL Positiva/terapia , Transfusão de Leucócitos , Pancitopenia/etiologia , Adulto , Doença Crônica , Terapia Combinada , Feminino , Fator Estimulador de Colônias de Granulócitos e Macrófagos/uso terapêutico , Humanos , Hidroxiureia/uso terapêutico , Interferon alfa-2 , Pancitopenia/terapia , Proteínas Recombinantes/uso terapêutico , Indução de Remissão , Terapia de SalvaçãoRESUMO
A 25-year-old woman developed an immunoblastic lymphoma 9 years after HLA-identical allogeneic bone marrow transplantation for T-cell acute lymphoblastic leukaemia in second remission. The B-cell origin of the second malignancy was confirmed by gene rearrangement studies. Despite continued donor engraftment, two separate genotypic analyses identified the lymphoma to be of recipient origin. This is the longest latency of a post-transplant recipient lymphoma yet reported and illustrates that recipient B-cells may survive the transplant conditioning regimen and undergo malignant transformation in the presence of donor haemopoiesis.
Assuntos
Transplante de Medula Óssea/efeitos adversos , Leucemia-Linfoma de Células T do Adulto/cirurgia , Linfoma de Células B/etiologia , Segunda Neoplasia Primária/etiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/etiologia , Adolescente , Southern Blotting , DNA de Neoplasias/análise , Feminino , Rearranjo Gênico/fisiologia , Humanos , Linfoma de Células B/genética , Segunda Neoplasia Primária/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/genéticaRESUMO
Between February 1988 and January 1990, 35 patients underwent allogeneic bone marrow transplantation (BMT) from unrelated donors using measures routinely employed for matched related donors. Median patient age was 34 years (range 2-49). Thirty-two patients had hematologic malignancies, including chronic myelogenous leukemia (CML) in 16; three patients had severe aplastic anemia. Donor-patient pairs were matched at the HLA loci tested serologically (HLA-A, -B, -DR) in 29 cases; mixed leukocyte culture results were variable but often reactive. Five patients died prior to day +28 without evidence of myeloid engraftment, and one patient developed fatal graft failure several months after initial engraftment. Acute graft-versus-host disease (GVHD) occurred in 77% (95% confidence interval [CI] 60-90%) of all patients, and GVHD contributed to the death of 10 patients. Fatal regimen-related toxicity occurred in four patients and another died due to neurologic complications of a process that resembled the hemolytic-uremic syndrome. Two acute leukemia patients relapsed, and a CML patient was found to have a localized non-Hodgkin's lymphoma at necropsy. As of 1 June 1991, 14 patients are alive and in remission at a median follow-up of 1.9 years (range 1.5-3.3); all except one have normal performance scores. The 2-year actuarial event-free survival for all patients is 40% (95% CI 24-56%). Proportional hazards analysis revealed favorable significance for female patient sex, less advanced disease status and shorter interval from diagnosis to BMT. While unrelated-donor transplants need not necessarily duplicate the results of related-donor transplants to be of benefit, the event-free survival in this series was roughly similar to that expected in the related-donor situation, with the high transplant-related mortality somewhat offset by a low recurrence rate. Further studies using unrelated donors, employing new methods of preventing transplant-related complications, are indicated.
Assuntos
Transplante de Medula Óssea/imunologia , Doadores de Tecidos , Transplante Homólogo/imunologia , Doença Aguda , Adolescente , Adulto , Anemia Aplástica/cirurgia , Transplante de Medula Óssea/efeitos adversos , Transplante de Medula Óssea/normas , Canadá , Criança , Pré-Escolar , Feminino , Doença Enxerto-Hospedeiro/epidemiologia , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/mortalidade , Antígenos HLA/imunologia , Histocompatibilidade/imunologia , Humanos , Incidência , Leucemia/cirurgia , Leucemia Mielogênica Crônica BCR-ABL Positiva/cirurgia , Masculino , Pessoa de Meia-Idade , Projetos PilotoRESUMO
Polymyositis developed in a patient who had had bone marrow transplants for the treatment of acute myeloid leukemia. There was no previous evidence of graft-versus-host disease. Polymyositis has previously been reported to be associated with graft-versus-host disease; this article suggests that polymyositis may represent its sole manifestation.
Assuntos
Doença Enxerto-Hospedeiro/diagnóstico , Miosite/diagnóstico , Adulto , Transplante de Medula Óssea/efeitos adversos , Doença Crônica , Doença Enxerto-Hospedeiro/complicações , Doença Enxerto-Hospedeiro/patologia , Humanos , Masculino , Músculos/patologia , Miosite/complicações , Miosite/patologia , Síndrome de Sjogren/complicaçõesRESUMO
Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare cause of thrombocytopenia. Since it is a syndrome of diverse etiologies, the optimal treatment is often uncertain. In a patient with longstanding AATP, a complete remission was obtained with antithymocyte globulin.
Assuntos
Megacariócitos/patologia , Trombocitopenia/patologia , Adulto , Soro Antilinfocitário/uso terapêutico , Medula Óssea/patologia , Feminino , Humanos , Contagem de Plaquetas , Trombocitopenia/sangueAssuntos
Proteínas Sanguíneas/deficiência , Proteínas de Membrana/deficiência , Espectrina/deficiência , Esferocitose Hereditária/sangue , Adulto , Anquirinas , Proteínas Sanguíneas/análise , Estabilidade de Medicamentos , Membrana Eritrocítica/análise , Feminino , Humanos , Masculino , Proteínas de Membrana/análise , Pessoa de Meia-Idade , Espectrina/análiseRESUMO
The cause of the renal failure that occurs in approximately 20 percent of patients following allogeneic bone marrow transplantation is poorly understood. A patient is described in whom acute renal failure occurred one week after allogeneic bone marrow transplantation. The onset of the renal failure was associated with the demonstration of anti-Lewis antibodies in the patient's serum, which could only have been derived from donor lymphocytes. Recovery of renal function coincided with the disappearance of the Lewis antibody. It is postulated that Lewis incompatibility between graft and host tissue may have contributed to the renal failure in this patient and that incompatibility associated with determinants present on renal cells may account for other instances of acute renal failure following allogeneic bone marrow transplantation.
Assuntos
Injúria Renal Aguda/etiologia , Incompatibilidade de Grupos Sanguíneos/complicações , Transplante de Medula Óssea , Antígenos do Grupo Sanguíneo de Lewis/genética , Transplante Homólogo/efeitos adversos , Adolescente , Feminino , Humanos , FenótipoAssuntos
Síndrome Hemolítico-Urêmica/sangue , Púrpura Trombocitopênica Trombótica/sangue , Difosfato de Adenosina/farmacologia , Adulto , Complexo Antígeno-Anticorpo/metabolismo , Ácidos Araquidônicos/metabolismo , Coagulação Sanguínea , Plaquetas/fisiologia , Vasos Sanguíneos/imunologia , Vasos Sanguíneos/patologia , Criança , Pré-Escolar , Endotélio/imunologia , Endotélio/patologia , Epoprostenol/biossíntese , Síndrome Hemolítico-Urêmica/fisiopatologia , Síndrome Hemolítico-Urêmica/terapia , Humanos , Rim/irrigação sanguínea , Troca Plasmática , Adesividade Plaquetária , Agregação Plaquetária , Púrpura Trombocitopênica Trombótica/fisiopatologia , Púrpura Trombocitopênica Trombótica/terapia , Trombina/farmacologia , Tromboxano A2/biossínteseRESUMO
The membrane deformability of erythrocytes from normal and dystrophic mice was determined using a flow channel technique whereby erythrocytes attached to the floor of a parallel plate channel were deformed by fluid shear forces. A nonlinear stress-strain experimental behavior was observed for both populations of erythrocytes which was best described with a polynormal expression: tau s = a epsilon x + [b epsilon x3/2 epsilon x + 1]. A comprehensive statistical analysis of the data indicated that a large percentage of the variance of the data was due to the experimental design. Furthermore, the 2 populations of cells were different in terms of the strain-stress relationship which best fitted the data, i.e., epsilon x = alpha tau s + beta tau s2 + gamma tau s3. Up to a shear stress of 5.5 dyn/cm2, where 95% of the data points were found, the dystrophic erythrocytes were slightly but significantly more deformable than the normal erythrocytes.
Assuntos
Membrana Eritrocítica/ultraestrutura , Eritrócitos/ultraestrutura , Distrofia Muscular Animal/sangue , Animais , Elasticidade , Camundongos , Camundongos Endogâmicos C57BL , Estresse MecânicoRESUMO
The recognition, investigation, diagnosis, and treatment of hemolytic anemia are reviewed on the basis of a classification of the causes of hemolysis according to whether they are disorders of the membrane, hemoglobin, or metabolism of the erythrocyte; congenital or familial or acquired; and intrinsic or extrinsic.