Suppr超能文献

[自身免疫性慢性活动性肝炎与环磷酰胺治愈的获得性红细胞生成减少症的关联]

[Association of autoimmune chronic active hepatitis and acquired erythroblastopenia cured by cyclophosphamide].

作者信息

Trinchet J C, Lacombe C, Islam S, Casadevall N, Varet B, Poupon R

出版信息

Gastroenterol Clin Biol. 1986 Dec;10(12):841-4.

PMID:3100375
Abstract

To the best of our knowledge this is the reported first case of the successive occurrence in the same patient of chronic active hepatitis and acquired pure red cell aplasia, both probably of autoimmune origin. The diagnosis of autoimmune hepatitis was based on the presence of characteristic lesions at the examination of the liver biopsy specimen, high titer of anti-smooth muscle antibodies in the serum, and remission obtained by steroid therapy. Erythroid aplasia, which appeared during the course of this treatment, was revealed by a regenerative anemia (4.4 g Hb/100 ml) and proved by bone marrow aspiration and biopsy. In vitro bone marrow culture was normal, suggesting the in vivo presence of an inhibitor of erythroblastic differentiation. Red cell aplasia was cured by cyclophosphamide (100 mg/day during 56 days). No recurrence was noted until the death of the patient, which occurred one year later, due to hepatic cholangiocarcinoma. Action of cyclophosphamide on the pure red cell aplasia suggested the immune origin of this disease. The liver and the bone marrow erythroid lineage have probably been the successive targets of immunologic dyscrasia.

摘要

据我们所知,这是首次报道同一患者先后发生慢性活动性肝炎和获得性纯红细胞再生障碍性贫血的病例,两者可能均起源于自身免疫。自身免疫性肝炎的诊断依据为肝活检标本检查发现特征性病变、血清中抗平滑肌抗体滴度高以及类固醇治疗取得缓解。在该治疗过程中出现的红细胞再生障碍,表现为再生障碍性贫血(血红蛋白4.4g/100ml),经骨髓穿刺和活检得以证实。体外骨髓培养正常,提示体内存在红细胞系分化抑制剂。红细胞再生障碍经环磷酰胺(56天内每日100mg)治愈。直至患者一年后因肝胆管癌死亡,均未观察到复发。环磷酰胺对纯红细胞再生障碍的作用提示该病的免疫起源。肝脏和骨髓红细胞系可能相继成为免疫失调的靶器官。

相似文献

4
Diagnosis and management of acquired pure red cell aplasia.获得性纯红细胞再生障碍性贫血的诊断与管理
Hematol Oncol Clin North Am. 2009 Apr;23(2):249-59. doi: 10.1016/j.hoc.2009.01.009.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验