Successful treatment with recombinant thrombomodulin in transplant-associated thrombotic microangiopathy following HLA-haploidentical transplantation

• Published in: Rinshō ketsueki Vol. 60; no. 11; p. 1560
• Main Authors: Otsuka, Yasuyuki, Kondo, Tadakazu, Nomura, Ryosuke, Yamashita, Kouhei, Takaori-Kondo, Akifumi
• Format: Journal Article
• Language: Japanese
• Published: Japan 2019
• Subjects: Adolescent; Female; Hematopoietic Stem Cell Transplantation - adverse effects; HLA Antigens; Humans; Thrombomodulin - therapeutic use; Thrombotic Microangiopathies - drug therapy; Thrombotic Microangiopathies - etiology; Transplantation, Haploidentical - adverse effects; HLA-haploidentical transplantation; Transplantation-associated thrombotic microangiopathy; Recombinant human soluble thrombomodulin
• ISSN: 0485-1439
• DOI: 10.11406/rinketsu.60.1560

In March 2009, a 17-year-old woman was first diagnosed with acute myelogenous leukemia and myelodysplasia-related changes. She underwent chemotherapy and allogeneic hematopoietic stem cell transplantation, which resulted in complete remission. However, she experienced relapse, and remission was achieved each time with repeated transplantation. In September 2014, a human leukocyte antigen (HLA)-haploidentical transplantation, which was the fifth allogeneic transplantation, was performed to treat the third relapse. Platelet transfusion refractoriness, hemolytic anemia with schistocytes, and renal dysfunction were observed from approximately the day of engraftment; therefore, transplantation-associated thrombotic microangiopathy (TA-TMA) was diagnosed. Recombinant human soluble thrombomodulin (rTM) was administered, and fresh-frozen plasma (FFP) was infused; this resulted in gradual improvement of TA-TMA. Treatment with rTM and FFP was discontinued on the 70th day after transplantation. Because the HLA-haploidentical transplantation was the fifth allogeneic transplantation, the risk of aggravation of TA-TMA was very high. Combined treatment with rTM and FFP, however, resulted in improvement of TA-TMA. Further investigation of similar cases is necessary for clarifying the usefulness of rTM for TA-TMA.