骨髄移植後溶血性尿毒症症候群の２例

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  • Two Cases of Hemolytic Uremic Syndrome following Bone Marrow Transplantation.

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Two cases of hemolytic uremic syndrome (HUS) following allogeneic bone marrow transplantation (BMT) are presented. Case 1 is a 14-year-old boy with acute leukemia who underwent allogeneic BMT from an HLA-matched sibling. His conditioning regimen was total body irradiation, L-PAM and thiotepa, and graft versus host disease (GVHD) prophylaxis consisted of short-term methotrexate (MTX) and cyclosporin A (CyA).At 7 months after BMT, he was admitted with edema on the lower extremities, hypertension, proteinuria, microhematuria and hemolytic anemia. He was diagnosed as having HUS. By stopping CyA administration, symptoms disappeared within 4 weeks. Case 2 is a 13-year-old girl with severe aplastic anemia who underwent allogeneic BMT from an HLA-matched unrelated donor. Her conditioning regimen was thoracoabdominal irradiation, cyclophosphamide and busulfan. GVHD prophylaxis consisted of short-term MTX and CyA. At 7 months after BMT, she showed pancytopenia, hemolytic anemia, proteinuria, microhematuria and hypertension.Similar to Case 1, administration of CyA was discontinued and anticoagulant therapy was employed with rapid improvement of clinical and laboratory findings. Although various factors such as chemotherapy, irradiation and infections are considered to be causal agents, the pathogenesis of HUS following BMT is still unknown. In the present cases, irradiation and viral infections might be involved in the pathogenesis of HUS.

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