Yohannan Binoy, Omo-Ogboi Allen C, Tammisetti Varaha S, Rios Adan
Division of Hematology and Oncology, McGovern Medical School, The University of Texas Health Science Center at Houston, Huston, TX, USA.
Department of Pathology and Laboratory Medicine, McGovern Medical School, The University of Texas Health Science Center at Houston, Huston, TX, USA.
J Hematol. 2022 Dec;11(6):216-222. doi: 10.14740/jh1049. Epub 2022 Dec 1.
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.
自身免疫性肝炎（AIH）是一种罕见的免疫介导性疾病，主要见于女性，由多种环境因素引发。极少数情况下，AIH可由潜在的恶性肿瘤引发。我们报告了一名60多岁的女性，其肝脏生化检查结果明显异常。血清学检查抗平滑肌抗体呈阳性，肝脏活检确诊为AIH。在住院期间，她发生了脓毒症和急性肾衰竭，需要透析支持。血清蛋白电泳（SPEP）显示单克隆IgG κ蛋白为1.92 g/dL，骨髓活检显示有7%的克隆性浆细胞。骨骼检查发现溶骨性病变，确诊同时存在多发性骨髓瘤（MM）。鉴于她的肝脏化学指标明显异常，我们决定先治疗AIH，并在她的临床状况改善后，使用类固醇（一种重要的抗骨髓瘤疗法）作为过渡，以便对MM进行特异性治疗。她接受了口服泼尼松和硫唑嘌呤治疗AIH。一个月后，肝脏生化检查结果显著改善，随后她开始口服伊沙佐米、来那度胺和地塞米松。她接受了针对腰椎（L2）、左股骨和坐骨病变的姑息性放疗。该病例凸显了AIH与MM罕见的同时发生情况，其潜在机制尚不清楚。
