Advanced stage mycosis fungoides (MF) is incurable with conventional treatments. High-dose chemo-radiotherapy with autologous stem cell transplantation has induced remissions in a small number of MF patients, but this modality is limited by a very high relapse rate. Allogeneic stem cell transplantation (Allo SCT) for MF is theoretically attractive, as there is no contamination of the graft by malignant cells, and because of the possibility of a graft-versus-tumor (GVT) effect. A single previously reported patient in the literature treated with Allo SCT for MF, relapsed on day 70 post-transplant. We describe a 37-year old woman with rapidly progressive stage IV ME Over 90Â°/a of her skin was covered with indurated, erythematous serpiginous pruritic plaques. Chronically infected ulcerations were present on the skin folds, breast, neck and forehead. She had 5% circulating Sezary cells, and bilateral axillary and inguinal lymphadonopathy by CT scans confirmed by biopsy. Her disease progressed over two years, despite treatment with topical corticosteroids, IFN, topical BCNU, IL-2, CHOP and 2-CdA. Prior to initiating the preparative regimen for Allo SCT, total skin electron beam (TSEB) therapy (1,600 cGy) was administered to reduce the skin ulceration and improve the skin barrier function. The patient received total body irradiation (1,320 cGy) and cyclophosphamide (120 mg/kg), followed by infusion of unmanipulated, G-CSF-mobilized peripheral blood stem cells from an HLA-identical sister. Graft-versus-host (GVHD) disease prophylaxis consisted of methotrexate and cyclosporine. She engrafted on Day +16 when her absolute neutrophil count was 720, hematocrit; 35, and platelet count 152,000. Grade II skin and gut GVHD developed on day+21, which responded to corticosteroids. A skin biopsy on Day +76 showed improvement of GVHD without any evidence of ME The marrow was normocellular, and 100 % of donor origin. Serial photographs documented that by day +85, all the MF skin lesions had resolved and serial CT scans showed resolution of lymphadenopathy. She remains in continuous complete unmaintained remission 1-year post-transplant, with limited chronic skin and gut GVHD. This represents the first report of durable unmaintained complete remission of refractory MF by Allo SCT. Although more patients and longer follow-up are needed, the results of this one patient suggest that Allo SCT should be considered as a treatment option in younger patients with advanced stage refractory MF. In the design of the transplant preparative regimen, upfront TSEB therapy might contribute to the clinical outcome since it may reduce skin ulceration and improve skin barrier function.
Full conference title:
American Society of Hematology 41 st Annual Meeting
- ASH 41st (2000)