作者: R. Storb , E. D. Thomas , C. D. Buckner , R. A. Clift , F. L. Johnson
DOI: 10.1182/BLOOD.V43.2.157.157
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摘要: Twenty-four patients with severe aplastic anemia, 14 due to unknown cause, four associated hepatitis, drug or chemical related, one paroxysmal nocturnal hemoglobinuria, and possibly a Fanconi syndrome did not show spontaneous recovery after 2-24 mo of conventional therapy. Eleven were infected nine refractory random platelet transfusions at the time admission. All received marrow grafts from HL-A identical siblings. Eighteen conditioned for grafting by cyclophosphamide (CY), 60 mg/kg on each 4 successive days, 6 1000 rad total body irradiation (TBI). intermittent methotrexate (MTX) therapy within first 100 days postgrafting modify graft-versus-host disease (QvHD). One patient died day congestive heart failure related CY cardiac toxicity. septicemia. 24 without engraftment. Twenty-one showed prompt hemopoietic engraftment indicated rising blood counts, return cellularity, in most instances confirmed genetic markers. Four rejected graft 33, 41, 51, 67. between 46 86 GvHD. cytomegalovirus infection 91. chronic active hepatitis 427 causes. are alive GvHD more than 141, 144, 163, 186, 189, 255, 344, 472, 641, 746, 823 grafting, ten have returned normal activity. These results that stem cells will repopulate anemia demonstrate long-term stable chimerism is possible man. They suggest complete an matched sibling should be undertaken before major infections refractoriness complicate their course.