同種骨髄移植を施行した再生不良性貧血の一症例

A Case of Severe Aplastic Anemia Treated with Allogeneic Bone Marrow Transplantation

Creators Yamada Katsunori

Creators Shinohara Kenji

Creators Satoh Yutaka

Creators Yoshizaki Yoshiki

Creators Inoue Masamitsu

Creators Fujii Yasuhiko

Creators Kaneko Toshio

Creators Igarashi Mie

Creators Fujita Kyoko

Creators Kajii Tadashi

Creators Matsumoto Noboru

A 14-year-old female with severe aplastic anemia received allogeneic bone marrow transplantation (BMT) from HLA-identical brother. She was pre-conditioned with intravenous cyclophosphamide (50mg/kg/day, for 4 days) and low dose total body irradiation (300 rad) followed by infusion of bone marrow cells (3.5×10^<10> nucleated cell and 5.3×10^6 CFU-C). The reconstitution of hematopoiesis appeared on day 17 and engraftment was certified by an analysis of sex chromosomes on day 21. This engraftment, however, was unsustained because of the late rejection charactarized by bicytopenia occurred on day 50. Severe acute graft versus host disease (GVHD) of grade IV appeared after BMT. Skin lesion developed on day 10 and extended to generalized erythroderma with epidermal necrosis. Liver and enteric lesions developed on day 34 as 11mg/dl of hyperbilirubinemia and 3000ml/day of diarrhea, respectively. High grade fever persisted during her hospital course and she died of intracranial hemorrhage on day 58. Autopsy findings revealed total denudation of epithelium of skin and intestine, extensive hepatocellular necrosis and small bile duct injury, and disseminated cytomegalovirus infection.

[ISSN]0513-1731

[NCID]AN00243156