RESUMO
PURPOSE: To demonstrate the efficacy of the UVAR XTS Photopheresis System and evaluate health-related quality of life in patients with early-stage mycosis fungoides (MF). PATIENTS AND METHODS: Extracorporeal photopheresis was administered 2 days every 4 weeks for 6 months. Patients with partial responses by skin weighted assessment continued for 6 months; nonresponders added oral bexarotene and/or interferon α. Health-related quality of life was assessed at baseline and every 3 months with 3 validated tools. RESULTS: Nineteen patients with early-stage MF (7 men, 12 women; 16 white, 3 African Americans) with median age of 63.5 years (range, 46-85 years) participated. Their stages were IA (n = 3), IB (n = 14), and IIA (n = 2). The overall response rate for extracorporeal photopheresis (ECP) alone, was 42% (8/19; including 7 partial response, 1 complete response), with a median of 12 ECP sessions (range, 3-32) given over a median of 12 months (3-32 months) and with an overall duration of response of 6.5 months (range, 1-48 months). Seven patients with stable disease at 3 months received additional bexarotene (3/5; 1 complete response) or bexarotene plus interferon α (1/2), and 4 (57%) of 7 responded. Treatment-related adverse effects were limited to those expected with interferon (fatigue, nausea, vomiting, and diarrhea), or with hypertriglyceridemia and bexarotene. Trends in health-related quality of life indicated an improvement in emotional scores over time. CONCLUSIONS: ECP is effective for patients with early-stage MF alone or in combination with biologic response modifiers with low toxicity and improved quality of life.
Assuntos
Micose Fungoide/terapia , Fotoferese , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/uso terapêutico , Bexaroteno , Contagem de Linfócito CD4 , Feminino , Humanos , Interferon-alfa/efeitos adversos , Interferon-alfa/uso terapêutico , Masculino , Pessoa de Meia-Idade , Micose Fungoide/imunologia , Micose Fungoide/patologia , Estadiamento de Neoplasias , Qualidade de Vida , Linfócitos T/imunologia , Tetra-Hidronaftalenos/efeitos adversos , Tetra-Hidronaftalenos/uso terapêutico , Resultado do TratamentoRESUMO
Neutrophilic dermatoses are a spectrum of diseases with varying presentations and similar clinical and histologic features. The association of neutrophilic dermatosis with celiac disease has not previously been described. We report a 43-year-old man with a history of diverticulitis who presented with mildly pruritic, ulcerating pustules of the dorsal hands, face, elbow, and neck, clinico-pathologically consistent with the neutrophilic dermatosis Sweet syndrome. Remission was achieved with prednisone taper, dapsone, and a gluten-free diet.
