Abstract
We investigated the long-term performance status of childhood recipients by means of a questionnaire because the quality of life in the long-term survivors following allogeneic hematopoietic stem cell transplantation remains unclear. Six cases of. secondary malignancy were reported. T-non Hodgkin lymphoma or myelodysplastic syndrome developed in the early period following transplantation and solid tumors such as brain tumors or lingual tumors in the late period. Chronic mucositis caused by the graft-versus-host disease (GVHD) might be involved in the carcinogenesis of the lingual tumor that occurred in a case of Fanconi anemia. Severe GVHD caused by HLA disparity and the administration of antithymocyte globulin were risk factors for the development of EB virus-associated lymphoproliferative disorder (EB-LPD). The deterioration of performance status caused by the development of chronic GVHD was reported in 46 cases. The development of bronchiolitis obliterans, arthropathy because of chronic GVHD of the skin, and encephalopathy related to the immunosuppressants were major sequels that influenced long-term performance status in pediatric survivors. It seems that the Karnofsky score used in this study may not reflect the patient's performance status because there is a discrepancy between the reported scores and symptoms. A new score system must be established to evaluate the quality of life from various aspects in long-term pediatric survivors.
