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异基因造血干细胞移植(hematopoietic stem cell transplantation,HSCT)后可出现各种自身免疫性疾病,但新发自身免疫性肝炎(de novo autoimmune hepatitis,de novo AIH)较为罕见。De novo AIH在成年患者中男性多于女性,儿童患者中男女各仅有1例报道,移植至发病间隔为135 d至2年不等,移植前基础病包括急慢性白血病、淋巴瘤、骨髓增生异常综合征和代谢性疾病等。De novo AIH无特异性症状或体征,主要临床表现为以转氨酶升高为主、伴或不伴黄疸的肝脏生化异常、免疫球蛋白G(immunoglobulin G,Ig G)升高、多种自身抗体阳性,组织学特点为汇管区淋巴浆细胞浸润、界面性肝炎、肝细胞坏死、纤维化等,对激素和硫唑嘌呤反应良好,但有复发倾向。
A variety of autoimmune diseases occur after hematopoietic stem cell transplantation (HSCT), but de novo autoimmune hepatitis (de novo AIH) is rare. De novo AIH has more males than females in adulthood and only 1 males and females in pediatric patients. The incidence of transplants was 135 days to 2 years. The pre-transplant basic diseases include acute and chronic leukemia, lymphoma, myeloproliferation Abnormal syndromes and metabolic diseases. De novo AIH no specific symptoms or signs, the main clinical manifestations of elevated aminotransferases, with or without jaundice liver biochemical abnormalities, immunoglobulin G (immunoglobulin G, Ig G) increased, a variety of autoantibodies positive Histological features of the portal area lymphoplasmacytic cell infiltration, interface hepatitis, hepatocyte necrosis, fibrosis, hormone and azathioprine good response, but there is a tendency of recurrence.