Extramedullary (EM) relapses after allogeneic hematopoietic stem cell transplantation (HSCT) are among the major causes of treatment failure. We retrospectively analyzed 174 patients with myeloid leukemia and myelodysplastic syndrome who underwent HSCT at the Hematology and Oncology Department of the Kyoto University Hospital between 1990 and 2009 to evaluate the incidence of EM relapses and determine its clinical features and appropriate treatment strategies. Of 53 patients who had a relapse after HSCT, 10 had an EM relapse, including 7 patients with a multiple-site EM relapse and 4 patients with an accompanied bone marrow (BM) relapse. Longer latency between HSCT and occurrence of relapse (486.5 versus 251 days) and a higher incidence of chronic graft-versus-host-disease (GVHD) before relapse (70% versus 26%) were observed for EM relapses than for BM relapses. Interestingly, EM relapses developed shortly after the intensification of the treatment for chronic GVHD (median 63 days, range: 28-339), indicating that immunosuppressive therapy triggers EM relapses. At 1 year, the survival rate after relapse was 46.7% for EM relapses. Therefore, effective management of EM relapses, frequently accompanied by chronic GVHD, is warranted.
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