Summary:We report a 51-year-old male with adult T cell leukemia (ATL) who received a BMT from an HLA-identical unrelated donor. The ATL proved refractory to chemotherapy, and he underwent BMT conditioned with CY/TBI. Complications of encephalitis of unknown origin were successfully treated with steroid therapy and the patient has been in CR for 16 months after BMT. Human T cell leukemia virus type 1 proviral DNA loads were reduced to undetectable levels in PBMC sampled 12 months after BMT. This encouraging result suggests that BMT from an unrelated donor should be considered for ATL even if the disease is refractory to chemotherapy. Bone Marrow Transplantation (2002) 30, 699-701. doi:10.1038/sj.bmt.1703702 Keywords: adult T cell leukemia; unrelated donor transplant; HTLV-1; encephalitis Adult T cell leukemia (ATL) is a peripheral CD4 + T cell malignancy endemic to southwestern Japan and the Caribbean basin, and human T cell leukemia virus type 1 (HTLV-1) plays a causative role in its development. The prognosis of ATL is extremely poor, with the median duration of overall survival being less than 1 year. Intensified chemotherapy and/or autologous stem cell transplantation have not yet improved this prognosis. 1,2 Although some successful cases of allogeneic stem cell transplantation (allo-SCT) have been reported, 3-5 HLA-matched and HTLV-1 seronegative siblings are available to only a small number of patients.We report a successful BMT from an unrelated donor in a patient with ATL refractory to various combination chemotherapy modalities. The patient has remained in CR for 16 months after BMT, with no detectable HTLV-1 infected cells. This case suggests that unrelated BMT can be used to successfully maintain long-term remission and cure even in this high-risk setting.
Case reportA 51-year-old Japanese male was initially referred to our hospital due to leukocytosis in March 1998. Systemic lymphadenopathy and splenomegaly were noted. The WBC count was 32.6 ϫ 10 9 /l. Differential count revealed 45% multilobulated abnormal lymphocytic cells positive for CD3, CD4 and CD25. Serum LDH was 815 IU/l (normal р420 IU/l), and calcium levels were normal. Antibody for HTLV-1 was positive, and monoclonal integration of HTLV-1 proviral DNA in PBMC was demonstrated using Southern blot analysis. A diagnosis of chronic type ATL was made according to the criteria of the Lymphoma Study Group. 6 The patient was followed without receiving chemotherapy. Abnormal lymphocytes in the peripheral blood decreased gradually during follow-up and had disappeared by July 1998, and subcutaneous nodules subsequently appeared on his chest and left thigh in December 1998. Biopsy of a chest nodule revealed T cell involvement, and he was treated with oral low-dose etoposide and localized irradiation to the nodules, resulting in a decrease in size. In March 2000, however, the patient presented with complaints of fever and general fatigue. Systemic lymphadenopathy and splenomegaly were noted. Laboratory data revealed leukocytosis (56.4 ϫ 10 9 /l) with 7...