Assuntos
Atrofia/etiologia , Infarto Encefálico/etiologia , Encéfalo/fisiopatologia , Hipotensão/complicações , Hipóxia-Isquemia Encefálica/etiologia , Mastocitose Sistêmica/complicações , Doença Aguda , Afasia/etiologia , Afasia/patologia , Afasia/fisiopatologia , Atrofia/patologia , Atrofia/fisiopatologia , Encéfalo/irrigação sanguínea , Encéfalo/patologia , Infarto Encefálico/patologia , Infarto Encefálico/fisiopatologia , Coma/etiologia , Coma/patologia , Coma/fisiopatologia , Progressão da Doença , Feminino , Antagonistas dos Receptores Histamínicos/uso terapêutico , Humanos , Hipotensão/fisiopatologia , Hipóxia-Isquemia Encefálica/patologia , Hipóxia-Isquemia Encefálica/fisiopatologia , Imageamento por Ressonância Magnética , Mastocitose Sistêmica/fisiopatologia , Pessoa de Meia-Idade , Quadriplegia/etiologia , Quadriplegia/patologia , Quadriplegia/fisiopatologia , Falha de Tratamento , Inconsciência/etiologia , Inconsciência/patologia , Inconsciência/fisiopatologia , Urticaria Pigmentosa/complicações , Urticaria Pigmentosa/patologia , Urticaria Pigmentosa/fisiopatologiaRESUMO
SUMMARY: Given the poor prognosis of patients with advanced cutaneous T-cell lymphoma and the high transplant-related mortality associated with conventional allogeneic bone marrow transplantation, we performed nonmyeloablative transplantation of allogeneic stem cells (ASCT) from HLA-identical siblings in three patients with this disease. All patients achieved full donor engraftment, clearance of clonal T cells leading to durable complete remissions but experienced high incidence of infections, which proved fatal in one case. These results suggest that nonmyeloablative ASCT is a novel and potentially curative therapy for patients with advanced T-cell lymphomas who have a histocompatible sibling.
Assuntos
Antifúngicos/uso terapêutico , Linfoma Cutâneo de Células T/complicações , Micose Fungoide/terapia , Transplante de Células-Tronco , Adulto , Feminino , Rearranjo Gênico da Cadeia gama dos Receptores de Antígenos dos Linfócitos T , Mobilização de Células-Tronco Hematopoéticas , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Micose Fungoide/tratamento farmacológico , Micose Fungoide/patologia , Reação em Cadeia da Polimerase , Receptores de Antígenos de Linfócitos T gama-delta/genética , Transplante de Células-Tronco/efeitos adversos , Quimeras de Transplante/imunologia , Transplante HomólogoRESUMO
Thromboembolic phenomena have been described in patients with thalassaemia intermedia and major, although there are relatively few epidemiological data on the overall frequency of these complications. To obtain more insight into the risk and mechanism of venous thromboembolism in thalassaemia, the aims of this study were: (i) to establish retrospectively the prevalence of thromboembolic events in a large group of adults with thalassaemia intermedia and major during a follow up period of 10 years; (ii) to measure in subgroups of these patients sensitive markers of activation of coagulation and fibrinolysis enzymes; and (iii) to look for possible procoagulant mechanisms. A high prevalence of thromboembolic events was found, particularly in splenectomized patients with thalassaemia intermedia (29%). These patients had high plasma levels of markers of coagulation and fibrinolysis activation. Furthermore, thalassaemic red cells and erythroid precursors from splenectomized patients with thalassaemia intermedia had an enhanced capacity to generate thrombin. To evaluate the role of splenectomy per se on procoagulant activity, we evaluated the capacity to form thrombin in healthy individuals who had been splenectomized for trauma. They produced the same amount of thrombin as non-splenectomized controls. In conclusion, the results of this study show the existence of a hypercoagulable state in splenectomized patients with thalassaemia intermedia and that their red and erythroid cells are capable of acting as activated platelets in thrombin generation.