RESUMO
BACKGROUND: Extracorporeal photopheresis (ECP) as a part of multimodality therapy, is one of the treatments for Sézary syndrome (SS) and advanced stage mycosis fungoides (MF). This study aims to describe cutaneous and peripheral blood responses of patients with MF and SS who received multimodality therapy. METHODS: In this cross-sectional retrospective study, patients with MF or SS who received ECP treatment in combination with at least one additional systemic treatment between 2011 and 2018 were included. ECP consisted of a two-session cycle every 2 to 4 weeks. Cutaneous and blood responses were evaluated with updated criteria. RESULTS: Twenty-eight patients (11 (39%) with MF and 17 (51%) with SS) were included. Their median age at diagnosis was 63 (57-67) years. The median number of treatments before ECP was 2 (1-3). Seven out of 11 patients with MF (63%) underwent an assessment of cutaneous response. Five patients (70%) presented a partial response; 1 (15%), stable disease, and 1 (15%) progressive disease. Thirteen of the 17 patients with SS (76%) underwent evaluation. One patient (8%) presented a complete cutaneous response; 6 (46%), a partial response; 5 (38%), stable disease; and 1 (8%), progressive disease. None of them relapsed during the study period in both groups. No ECP-related adverse effects occurred during the study. CONCLUSION: Most patients with SS and MF who underwent multimodality therapy with ECP had favorable cutaneous and blood response. It is safe to combine ECP with other treatments. Studies with large numbers of patients are necessary to assess the effects of ECP on patient survival.
Assuntos
Linfoma Cutâneo de Células T/terapia , Fotoferese/métodos , Idoso , Argentina , Terapia Combinada , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/terapia , Estudos Retrospectivos , Síndrome de Sézary/terapia , Neoplasias Cutâneas/terapia , Resultado do TratamentoRESUMO
Graft-versus-host disease (GVHD) is a major complication of allogeneic hematopoietic stem cell transplants (allo-HSCT) associated with significant morbidity and mortality. The earliest and most common manifestation is cutaneous graft-versus-host disease. This review focuses on the pathophysiology, clinical features, prevention and treatment of cutaneous graft-versus-host disease. We discuss various insights into the disease's mechanisms and the different treatments for acute and chronic skin graft-versus-host disease.
Assuntos
Doença Enxerto-Hospedeiro/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Dermatopatias/etiologia , Doença Crônica , Exantema/etiologia , Exantema/terapia , Glucocorticoides/uso terapêutico , Doença Enxerto-Hospedeiro/terapia , Humanos , Fotoferese/métodos , Dermatopatias/terapiaRESUMO
Abstract Graft-versus-host disease (GVHD) is a major complication of allogeneic hematopoietic stem cell transplants (allo-HSCT) associated with significant morbidity and mortality. The earliest and most common manifestation is cutaneous graft-versus-host disease. This review focuses on the pathophysiology, clinical features, prevention and treatment of cutaneous graft-versus-host disease. We discuss various insights into the disease's mechanisms and the different treatments for acute and chronic skin graft-versus-host disease.
Assuntos
Humanos , Dermatopatias/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Doença Enxerto-Hospedeiro/etiologia , Dermatopatias/terapia , Doença Crônica , Fotoferese/métodos , Exantema/etiologia , Exantema/terapia , Glucocorticoides/uso terapêutico , Doença Enxerto-Hospedeiro/terapiaRESUMO
Sézary syndrome (SS) is an unusually aggressive T- cell lymphoma characterized by the triad of erythroderma, the presence of more than 1,000 Sézary cells in peripheral blood and lymphadenopathies. It is accompanied by generalized pruritus and poor quality of life. The management of SS depends on its stage, patient comorbidities, and treatment availability. Extracorporeal photopheresis (ECP) is the first line of treatment for patients with T-cell lymphomas in stage IVA1, IVA2 or SS. This treatment comprises three phases: leukapheresis, photoactivation and subsequent reinfusion of lymphocytes. As it is an immunomodulatory therapy it does not produce generalized immunosuppression. We report a 76 year-old male with SS stage IIIb initially treated with 12 sessions of ultraviolet phototherapy without response. After 10 well-tolerated sessions of ECP, itching and skin lesions eventually disappeared.
Assuntos
Idoso , Humanos , Masculino , Fotoferese/métodos , Síndrome de Sézary/terapia , Neoplasias Cutâneas/terapia , Biópsia , Fibroblastos/patologia , Citometria de Fluxo , Prurido/patologia , Indução de Remissão/métodos , Síndrome de Sézary/patologia , Neoplasias Cutâneas/patologiaRESUMO
Sézary syndrome (SS) is an unusually aggressive T- cell lymphoma characterized by the triad of erythroderma, the presence of more than 1,000 Sézary cells in peripheral blood and lymphadenopathies. It is accompanied by generalized pruritus and poor quality of life. The management of SS depends on its stage, patient comorbidities, and treatment availability. Extracorporeal photopheresis (ECP) is the first line of treatment for patients with T-cell lymphomas in stage IVA1, IVA2 or SS. This treatment comprises three phases: leukapheresis, photoactivation and subsequent reinfusion of lymphocytes. As it is an immunomodulatory therapy it does not produce generalized immunosuppression. We report a 76 year-old male with SS stage IIIb initially treated with 12 sessions of ultraviolet phototherapy without response. After 10 well-tolerated sessions of ECP, itching and skin lesions eventually disappeared.
Assuntos
Fotoferese/métodos , Síndrome de Sézary/terapia , Neoplasias Cutâneas/terapia , Idoso , Biópsia , Fibroblastos/patologia , Citometria de Fluxo , Humanos , Masculino , Prurido/patologia , Indução de Remissão/métodos , Síndrome de Sézary/patologia , Neoplasias Cutâneas/patologiaRESUMO
El reconocimiento de la participación de mecanismos inmunológicos en la fisiopatología de muchas enfermedades ha estimulado el desarrollo de nuevas estrategias terapéuticas de inmunointervención como la fotoféresis, la radioféresis y la vacunación con células dendríticas. Las observaciones clínicas han mostrado efectos positivos y resultados alentadores en la utilización de estas terapias