造血干细胞移植后新发自身免疫性肝炎临床特点 诊断与治疗

Clinical manifestations, diagnosis and treatments of de novo autoimmune hepatitis after hematopoietic stem cell transplantation

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。

Autoimmune diseases may occur after allogeneic hematopoietic stem cell transplantation （HSCT）, about de novo autoimmune hepatitis （de novo AIH） is a rare complication after HSCT. This is a review about clinical manifestations, diagnosis and treatments of de novo AIH after HSCT. There were more male than female patients in adults, while only one case was reported in each of male and female pediatric patients. The interval between transplantation and de novo AIH onset ranged from 135 days to 2 years. The underlying diseases included acute and chronic myeloid leukemia, acute and chronic lymphocytic leukemia, Hodgkin and non-Hodg!dn＇s lymphoma, myelodysplastic syndrome and metabolic diseases. Clinical manifestations were characterized by elevated transaminase with or without jaundice, as well as elevated serum gammaglobulin （IgG） and positive autoantibodies. The histological features were plasma cells aggregations at portal areas, interface hepatitis, necro-inflammation and fibrosis. Patients had good responses to the treatment with corticosteroids and azathioprine, while the disease tended to relapse.