483
483
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第483章
DOI：
10.1097/01.ccm.0000551235.27758.8a
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发表时间：
2019
影响因子：
8.8
通讯作者：
A. Aggarwal
中科院分区：
文献类型：
--
作者：
N. Cherian;J.;K. Schwarzová;P. Mathur;A. Aggarwal
方法：一名 72 岁男性，患有继发 NASH 肝硬化的 ESLD，接受了 OLT。术中和术后过程均无异常。他接受甲基泼尼松龙诱导，并继续使用他克莫司和麦考酚酯进行免疫抑制。四周后，他出现腹泻、疲劳和呼吸急促的症状，到急诊科就诊。入院实验室显示严重全血细胞减少症，白细胞为 0.03 x103/μl，影像学显示腹部和胸膜积液。出现低血压和低氧血症后，他被送入外科重症监护室。患者的临床状况进一步恶化，出现精神错乱、少尿性肾损伤和呼吸功能不全。他需要机械通气和升压药支持。患者保留免疫抑制并给予非格司亭。在此期间，他出现了弥漫性红斑丘疹，尤其是毛囊周围的紫罗兰色丘疹。皮肤活检证实GVHD。鉴于他对非格司亭的反应不足，进行了骨髓活检，结果显示细胞性低下且发育不全，也与急性 GVHD 一致。初始治疗以大剂量甲基泼尼松龙和抗胸腺细胞球蛋白开始。在住院期间进行了多项嵌合研究作为治疗反应的标志。由于对胸腺球蛋白缺乏反应，我们决定试用乌特克单抗，这是一种常用于治疗骨髓移植后 GVHD 的药物。后续研究显示没有任何改善。最终，在家人将护理目标修改为舒适措施后，患者去世了。结果：由于 OLT 后 GVHD 的罕见性，尚无标准治疗方案。个体病例报告是提高初步临床怀疑、促进早期诊断并为有效治疗方式提供新见解的宝贵手段。
Methods: A 72-year-old male with ESLD secondary to NASH cirrhosis underwent an OLT. Intraoperative and postoperative course was unremarkable. He was given methylprednisolone for induction and immunosuppression was continued with tacrolimus and mycophenolate. He presented to the ED four weeks later with symptoms of diarrhea, fatigue and shortness of breath. Admission labs demonstrated severe pancytopenia with WBC 0.03 x103/µl and imaging showed abdominal and pleural fluid collections. He was admitted to the surgical ICU after developing hypotension and hypoxemia. Patient’s clinical condition further deteriorated as he developed confusion, oliguric kidney injury and respiratory insufficiency. He required mechanical ventilation and vasopressor support. Patient’s immunosuppression was held and filgrastim was given. During this time, he developed diffuse erythematous maculopapular rash, especially violaceous papules surrounding hair follicles. Skin biopsy confirmed GVHD. Given his inadequate response to filgrastim, a bone marrow biopsy was done and demonstrated hypocellularity with panhypoplasia also consistent with acute GVHD. Initial therapy was initiated with high-dose methylprednisolone and anti-thymocyte globulin. Multiple chimerism studies were performed during hospital course as a marker for treatment response. Due to a lack of response to thymoglobulin, we decided to trial ustekinumab, a medication often used for treatment of GVHD after bone marrow transplant. Follow-up studies showed no improvement. Ultimately, the patient passed away after family revised goals of care to comfort measures.Results: No standard treatment regimen for GVHD post OLT is available due to the rarity of the condition. Individual case reports are a valuable means of heightening initial clinical suspicion, fostering earlier diagnosis and providing new insights to effective treatment modalities.