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终末期肝病中严重血小板减少症的应对:移植前管理难题

Navigating Severe Thrombocytopenia in End-Stage Liver Disease: A Pre-transplant Management Dilemma.

作者信息

Jamal Fares, Elshaer Amani, Eslinger Cody, Naidu Sailendra, Dickson Rolland C, Padrnos Leslie J

机构信息

Hematology and Oncology, Mayo Clinic, Phoenix, USA.

Internal Medicine, Mayo Clinic, Phoenix, USA.

出版信息

Cureus. 2025 Jun 11;17(6):e85811. doi: 10.7759/cureus.85811. eCollection 2025 Jun.

Abstract

End-stage liver disease (ESLD) due to autoimmune causes may be complicated by immune thrombocytopenia (ITP), making liver transplantation challenging. We present a case of refractory ITP in a liver transplant candidate and outline therapeutic strategies used to manage critical thrombocytopenia. A 60-year-old woman with ESLD from primary biliary cholangitis and autoimmune hepatitis was evaluated for liver and kidney transplants. Her platelet count declined from 45 K/µL to 12 K/µL, prompting hematology involvement. Despite intravenous immunoglobulin (IVIG) and dexamethasone, the platelet response was limited. Rituximab had no effect, and splenectomy was too risky pre-transplant due to portal hypertension. Thrombopoietin receptor agonists were limited by thrombotic risk. Subdural hematomas further complicated her course. Ultimately, partial splenic artery embolization improved her platelet count to 25 K/µL, allowing combined liver-kidney transplantation with splenectomy. Postoperatively, her platelet count increased to 854 K/µL without complications. Managing refractory ITP in ESLD requires a multifaceted approach. While corticosteroids and IVIG are first-line therapies, options like thrombopoietin agonists pose thrombotic risks. Splenic artery embolization may be a viable strategy to increase platelet counts before transplantation. Further research is needed to guide treatment in this complex population.

摘要

自身免疫性病因导致的终末期肝病(ESLD)可能并发免疫性血小板减少症(ITP),这使得肝移植具有挑战性。我们报告一例肝移植候选者的难治性ITP病例,并概述用于管理严重血小板减少症的治疗策略。一名60岁患有原发性胆汁性胆管炎和自身免疫性肝炎所致ESLD的女性接受了肝和肾移植评估。她的血小板计数从45 K/µL降至12 K/µL,促使血液科介入。尽管使用了静脉注射免疫球蛋白(IVIG)和地塞米松,但血小板反应有限。利妥昔单抗无效,且由于门静脉高压,脾切除术在移植前风险太大。血小板生成素受体激动剂受血栓形成风险限制。硬膜下血肿使她的病情更加复杂。最终,部分脾动脉栓塞术将她的血小板计数提高到25 K/µL,从而得以进行肝肾联合移植并同时行脾切除术。术后,她的血小板计数升至854 K/µL,且无并发症。管理ESLD中的难治性ITP需要多方面的方法。虽然皮质类固醇和IVIG是一线治疗方法,但血小板生成素激动剂等选择存在血栓形成风险。脾动脉栓塞术可能是在移植前增加血小板计数的可行策略。需要进一步研究来指导这一复杂人群的治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5ff3/12254519/fe1828511a6b/cureus-0017-00000085811-i01.jpg

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