Chronic graft-versus-host disease in 52 patients: adverse natural course and successful treatment with combination immunosuppression.
Chronic graft-versus-host disease in 52 patients: adverse natural course and successful treatment with combination immunosuppression.
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DOI：
10.1182/blood.v57.2.267.bloodjournal572267
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发表时间：
1981-02
期刊：
影响因子：
20.3
通讯作者：
K. Sullivan;H. Shulman;R. Storb;Weiden Pl;R. Witherspoon;G. McDonald;M. Schubert;K. Atkinson;Thomas Ed
中科院分区：
文献类型：
--
作者：
K. Sullivan;H. Shulman;R. Storb;Weiden Pl;R. Witherspoon;G. McDonald;M. Schubert;K. Atkinson;Thomas Ed
在175例异基因骨髓移植的幸存者中，52例(30%)发展为慢性移植物抗软组织病(GVHD)。5例局限性慢性移植物抗宿主病的临床病程缓慢，仅累及皮肤和肝脏。47名患有广泛慢性GVHD的患者有类似于几种自身免疫性疾病的不利的多器官功能障碍。13例广泛性疾病患者(I组)未接受治疗，仅2例存活，Karnofsky评分-70%。死亡是由干燥综合征、肺和肝功能不全、硬皮病样皮肤病和痉挛引起的感染和发病造成的。另有13例(II组)接受中位数为8mo的泼尼松和/或短程抗胸腺细胞球蛋白治疗，3例无残疾存活。另21例(III组)给予泼尼松(1.0 mg/kg/q.o.d)。环磷酰胺、丙卡巴肼或硫唑嘌呤(均为1.5 mg/kg/d)，中位数为13mo。联合治疗耐受性良好，仅有轻微的骨髓毒性。III组治疗有效15例，一般4例，无反应死亡2例。硫唑嘌呤和泼尼松是最有效的治疗方案。12例III组患者全部停止治疗：GVHD复发5例(包括2例因复治死亡)，7例无GVHD，观察时间中位数为11(6~30)mo。移植后2~4年，仅I组III组幸存者残废，16例存活，Karnofsky评分70%~100%。因此，联合免疫抑制似乎对广泛性慢性移植物抗宿主病的不利自然病程有有利的影响，在某些情况下，还能阻止这一不利的自然病程。
Fifty-two of 175 (30%) survivors of allogeneic marrow transplantation developed chronic graft-versus-hose diseases (GVHD). Five with limited chronic GVHD had an indolent clinical course with involvement of only the skin and liver. Forty-seven with extensive chronic GVHD had an unfavorable multiorgan disorder that resembled several autoimmune diseases. Thirteen patients with extensive disease (group I) were not treated and only 2 survive with Karnofsky scores >- 70%. Mortality resulted from infections and morbidity from sica syndrome, pulmonary and hepatic insufficiency, scleroderma-like skin disease, and contractures. Another 13 (group II) received a median of 8 mo prednisone and/or a brief course of antithymocyte globulin, and 3 survive without disability. The other 21 (group III) were treated with a combination of prednisone (1.0 mg/kg/q.o.d.) and either cyclophosphamide, procarbazine, or azathioprine (all 1.5 mg/kg/day) for a median of 13 mo. Combination therapy was well tolerated with only modest myelotoxicity. Fifteen in group III had a good and 4 a fair response to treatment while 2 with no response died. Azathioprine and prednisone was the most effective regimen. All therapy has been discontinued in 12 group III patients: GVHD returned in 5 (including 2 who died in spite of retreatment) while 7 remain free of GVHD for a median of 11 (range 6-30) mo observation. Only I group III survivor is disabled and 16 of the original 21 are alive 2-4 yr after transplant with Karnofsky scores of 70%-100%. Thus, combination immmunosuppression appears to favorably affect and, in some cases, premanently arrest the adverse natural course of extensive chronic GVHD.