Allogeneic hematopoietic stem cell transplantation in mycosis\nfungoides
A 44-year-old white woman observed an erythema in the sternal region that progressed to\ninvolvement of the whole body in the form of urticarial plaques. The picture remained\nstable with topical and systemic steroids for eight years. Progression was observed with\nelevated and pruritic patches and plaques with purulent discharge from secondary\ninfection on an extensive portion of the body surface.\nWhen the patient came to our service, we performed a skin biopsy with\nimmunohistochemistry that revealed cutaneous lymphoproliferative disease compatible with\nanaplastic large T-cell lymphoma focally CD30+ or Histiocytic sarcoma. Bone marrow\nbiopsy was normal. CHOEP (cyclophosphamide, doxorubicin, etoposide, vincristine and\nprednisone) chemotherapy was initiated, totaling eight cycles, with minimal initial\nresponse in plaques and pruritus, but reappearing at the end of the eighth cycle. A new\nskin biopsy showed cutaneous T-cell lymphoma with epidermotropism, CD45+ and CD3+\n(suggesting MF). Staged as IIB (T3-N0-M0), with 80% affected area, and presenting the\nmost advanced lesions in the trunk, breasts and legs.\nSubcutaneous interferon 3,000,000U was initiated, three times a week, gradually\nincreasing to 12,000,000U and radiotherapy in tougher skin areas in the right groin.\nMajor improvement of the lesions was obtained with interferon, but this had to be\nsuspended because of non-hematological toxicity and disease progression. Gemcitabine,\n1000mg/m² per week, was initiated. There was a significant regression of lesions, but\nrecurrence was noticed at the end of the sixth cycle.\nHer condition progressively worsened despite partial and temporary improvements with the\nvarious treatment modalities. The patient's skin was diffusely infiltrated ( and ) with patches, plaques, and tumors. The patient was referred to Euryclides de\nJesus Zerbini Transplants Hospital, in São Paulo, and a reduced-intensity conditioning\n(RIC) allogeneic HLA matched sibling transplant was performed. After conditioning with\nfludarabine and melfalan she received peripheral blood stem-cells from her brother.\nGraft-versus-host-disease (GVHD) prophylaxis was done with mycophenolate mofetil and\ncyclosporine.\nEngraftment took place on D+11 and the patient was discharged from the hospital on D+14,\nwith complete remission. Skin biopsy of residual hyperpigmented patches () showed GVHD without evidence of lymphocyte\natypia. The patient has been in complete remission ( and ) for more than one year\nwithout treatment or any signs of GVHD, and peripheral blood mononuclear cells are 100%\ndonor.