硬皮病样慢性移植物抗宿主病3例

Clinical analysis of 3 patients with sclerodermatous chronic graft-versus-host disease

报告3例硬皮病样慢性移植物抗宿主病。患者均为男性,平均年龄48岁。因原发性血液系统疾病在化疗缓解后接受异体骨髓移植,移植后平均12.7个月出现全身弥漫性硬化或泛发性硬斑,硬化严格以手腕、踝部和颈部为界,未累及肢体远端和面部,无雷诺现象和毛细血管扩张。其中2例伴有不同程度的肺间质病变。3例患者抗核抗体ANA、类风湿因子RF、抗Scl-70抗体和抗着丝点抗体均为阴性,组织病理检查显示真皮网状层胶原纤维束增粗、密集和肿胀。3例患者均接受中等剂量的糖皮质激素治疗,其中2例治疗1个月后皮肤明显软化,另1例软化不明显。

We report three cases of sclerodermatous chronic graft-versus-host disease recruited from 2001 to 2002 in our department. All of these patients were male, with a mean age of 48 years. They underwent allogenic bone marrow transplantation (ABMT) due to hematological diseases. Sclerotic lesions appeared after ABMT on 12.7 month average. Sclerosis was generalized, but the extremities and the face were not involved. We found no Raynaud phenomenon or telangiectasis. ANA, RF, anti-Scl-70 and anti-centromere antibodies were negative. 2 patients presented with interstitial lung disease. Histopathology showed that in stratum reticulare collagen fibers were thickened, densified and swollen. 2 patients alleviated after the treatment with corticosteroid in various dosages, but no obvious melioration was found in third patient.