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.v57.2.267.267
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发表时间：
1981-01-01
期刊：
影响因子：
20.3
通讯作者：
THOMAS, ED
中科院分区：
文献类型：
--
作者：
SULLIVAN, KM;SHULMAN, HM;THOMAS, ED
175例异基因骨髓移植存活者中约有52例（30%）发生慢性移植物抗-宿主病（GVHD）。5例有限慢性GVHD患者的临床病程缓慢，仅累及皮肤和肝脏。47例广泛的慢性GVHD患者有类似于几种自身免疫性疾病的不利的多器官疾病。约13名患有广泛疾病的患者（I组）未接受治疗，只有2名存活，Karnofsky评分≥。百分之七十感染导致死亡，干燥综合征、肺和肝功能不全、硬皮病样皮肤病和挛缩导致发病。另外13例（组II）接受了中位数为8个月的治疗。强的松[PD]和/或抗胸腺细胞球蛋白的短期疗程，3例存活无残疾。另外21只（第III组）接受PD（1.0 mg/kg，每日4次）和环磷酰胺、甲基苄肼或硫唑嘌呤（均为1.5 mg/kg/天）联合治疗，中位时间为13个月。联合治疗耐受性良好，仅出现中度骨髓毒性。III组15例疗效良好，4例疗效尚可，2例无效死亡。硫唑嘌呤和PD是最有效的方案。12例III组患者停止所有治疗：5例GVHD复发（包括2例再次治疗后死亡），7例无GVHD，中位时间为11个月（范围6-30）。观察.只有I组III存活者被致残，并且最初的21例中有16例在移植后2-4年存活，Karnofsky评分为70%-100%。联合免疫抑制似乎有利地影响，并在某些情况下，永久阻止广泛的慢性GVHD的不良自然过程。
Some 52 of 175 (30%) survivors of allogeneic marrow transplantation developed chronic graft-vs.-host disease (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. Some 13 patients with extensive disease (group I) were not treated and only 2 survive with Karnofsky scores .gtoreq. 70%. Mortality resulted from infections and morbidity from sicca syndrome, pulmonary and hepatic insufficiency, scleroderma-like skin disease, and contractures. Another 13 (group II) received a median of 8 mo. prednisone [PD] and/or a brief course of antithymocyte globulin, and 3 survive without disability. The other 21 (group III) were treated with a combination of PD (1.0 mg/kg 4 times daily) and either cyclophosphamide, procarbazine, or azathioprine (all 1.5 mg/kg per day) for a median of 13 mo. Combination therapy was well tolerated with only modest myelotoxicity. In group III 15 had a good and 4 a fair response to treatment while 2 with no response died. Azathioprine and PD was the most effective regimen. All therapy was 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 disabeled and 16 of the original 21 are alive 2-4 yr after transplant with 70%-100% Karnofsky scores. Combination immunosuppression appears to favorably affect and, in some cases, permanently arrest the adverse natural course of extensive chronic GVHD.