Summary:Allogeneic hematopoietic stem cell transplantation should be considered as a therapeutic option for patients with generalized erythoderma or tumor stage MF. Indeed, the only curative option for MF may be an allogeneic transplant. Bone Marrow Transplantation (2000) 25, 111-113. Keywords: allogeneic hematopoietic stem cell transplant; mycosis fungoides; cutaneous T cell lymphoma Mycosis fungoides (MF) is a low-grade lymphoma that primarily manifests as cutaneous plaques or tumors. 1,2 Progression is accompanied by spread to lymph nodes and visceral organs. Histopathology reveals an epidermotropic lymphocytic infiltrate composed of atypical lymphocytes. 3 The atypical cells may also be seen in the peripheral blood and are characterized by their cerebriform or hyperconvoluted nuclear detail. MF cells are of the T cell lineage and usually have a CD4 ϩ CD45RO ϩ memory-helper phenotype. 4 The natural history of MF is usually indolent. Over many years MF may evolve from premalignant cutaneous lesions and scaly erythematous patches into palpable plaques and finally tumors. The mean survival for patients with early patch lesions may exceed 10-15 years. However, patients with generalized erythroderma or tumor at the time of presentation have a poorer prognosis with a median survival of 2-4 years. Early stage lesions are treated with psoralen and ultraviolet light A (PUVA), topical chemotherapy, extracorporeal photophoresis, interferon, and/or electron beam irradiation. 5,6 Advanced disease is treated with interferon, retinoids or combination chemotherapy regimens, with mixed results. 5,7 To our knowledge, we report the first case of reinduction of clinical and histologic remission following withdrawal of immunosuppressive medication after allogeneic hematopoietic stem cell transplantation, providing evidence for an immunologic graft-versus-MF effect.
Case reportA 27-year-old black woman was diagnosed with mycosis fungoides (stage T3 N1 M0). She had failed multiple prior therapies including PUVA, PUVA and interferon, six cycles of CHOP (cyclophosphamide, adriamycin, vincristine, prednisone), and two cycles of 9-aminocamptothecin. The patient's skin was diffusely infiltrated with patches, plaques and tumors. Lymphatic and visceral organs were involved. An allogeneic HLA matched sibling transplant was performed using an unmanipulated marrow and CD34 immunoselected blood graft. Conditioning was with cyclophosphamide (200 mg/kg) and total body irradiation (1200 cGy). The post-transplant course was complicated by acute graft-versus-host disease (grade II) that resolved on corticosteroid and cyclosporine immunosuppressive therapy.The patient remained in complete remission for 9 months when new plaques were noted over the right thigh and chest (Figure 1a). Relapse of MF was confirmed by skin biopsy (Figure 1b and c). Since there was no clinical evidence of GVHD, prophylactic immunosuppression with cyclosporine was discontinued. Within 1 month, the plaques resolved and were replaced by flat and scaly hypopigmented patches....