自身免疫性肝炎和多发性骨髓瘤的同步表现

IF 1.3 Q4 HEMATOLOGY Journal of hematology Pub Date : 2022-12-01 DOI:10.14740/jh1049
Binoy Yohannan, Allen C Omo-Ogboi, Varaha S Tammisetti, Adan Rios
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摘要

自身免疫性肝炎(AIH)是一种罕见的免疫介导疾病,主要见于女性,由各种环境因素引发。罕见的是,AIH可由潜在的恶性肿瘤引发。我们报告一位60多岁的妇女,她的肝脏生化检查明显异常。血清学抗平滑肌抗体阳性,肝脏活检证实AIH。在住院期间,她出现败血症和急性肾衰竭,需要透析支持。血清蛋白电泳(SPEP)显示单克隆IgG kappa蛋白为1.92 g/dL,骨髓活检显示7%的克隆浆细胞。她有溶解性病变骨骼调查证实诊断为共存多发性骨髓瘤(MM)。鉴于她的肝脏化学物质明显异常,我们决定先治疗AIH,并在她的临床情况好转后使用类固醇(一种重要的抗骨髓瘤治疗方法)作为桥梁,对MM进行特异性治疗。她口服强的松和硫唑嘌呤治疗AIH。1个月后,肝脏生化检查结果明显改善,开始口服伊唑唑米、来那度胺和地塞米松。她接受了腰椎(L2)、左股骨和坐骨病变的姑息性放疗。该病例突出了AIH和MM的罕见共存,其潜在机制尚不清楚。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Synchronous Presentation of Autoimmune Hepatitis and Multiple Myeloma.

Autoimmune hepatitis (AIH) is a rare immune-mediated disease predominantly seen in women and triggered by various environmental factors. Rarely, AIH can be triggered by an underlying malignancy. We report a woman in her 60s who presented with markedly abnormal liver biochemical tests. Serology was positive for anti-smooth muscle antibodies and a liver biopsy confirmed AIH. During the hospital course, she developed sepsis and acute renal failure requiring dialysis support. Serum protein electrophoresis (SPEP) showed a monoclonal IgG kappa protein of 1.92 g/dL and a bone marrow biopsy revealed 7% clonal plasma cells. She had lytic lesions on skeletal survey confirming the diagnosis of a coexisting multiple myeloma (MM). Given her markedly abnormal liver chemistries, we decided to treat the AIH first and use the steroids (an important anti-myeloma therapy) as a bridge to the specific treatment of the MM once her clinical condition improved. She was treated with oral prednisone and azathioprine for AIH. One month later, a marked improvement in liver biochemical test results was noted and she was started on oral ixazomib, lenalidomide and dexamethasone. She received palliative radiotherapy to the lumbar spine (L2), left femur, and ischium lesions. This case highlights a rare co-occurrence of AIH and MM, the underlying mechanism of which is unknown.

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来源期刊
Journal of hematology
Journal of hematology HEMATOLOGY-
自引率
0.00%
发文量
29
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