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患有哈里斯血小板综合征相关血小板减少症的活体肝供体:病例系列

Living Liver Donors With Harris Platelet Syndrome Related Thrombocytopenia: A Case Series.

作者信息

Gupta Prateek, Kanagavelu Rathnavel G, Sam Amal F, Rammohan Ashwin, Balasubramanian Balaji, Bibi Asma, Rajakumar Akila, Rela Mohamed

机构信息

The Institute of Liver Disease & Transplantation, Dr.Rela Institute & Medical Centre, Bharath Institute of Higher Education & Research, Chennai, India.

Department of Hematology and Clinical Pathology, Bharath Institute of Higher Education and Research, Chennai, India.

出版信息

J Clin Exp Hepatol. 2024 Nov-Dec;14(6):101475. doi: 10.1016/j.jceh.2024.101475. Epub 2024 Jul 1.

Abstract

Donor safety is of paramount importance for living-donor liver transplantation. Donor thrombocytopenia is viewed with caution and considered a relative contraindication for donations. Harris platelet syndrome (HPLS), a recently described cause of asymptomatic thrombocytopenia, is a subtype of inherited giant platelet disorders noted more commonly in the north-eastern part of India. Herein, we present our experience with living liver donors using HPLS. We retrospectively collected data from all patients with HPLS who underwent donor hepatectomies. Five patients were diagnosed as having HPLS. The mean intraoperative blood loss was 350 ml. On post-operative day 1, all patients had lactate levels of <2 mmol/L. None of the patients had any features of post hepatectomy liver failure, and the international normalized ratio was normalized by post-operative day 5. There needs to be awareness of the benign nature of HPLS. This would avoid unnecessarily ruling out suitable candidates who would otherwise yield an appropriate graft.

摘要

供体安全对于活体肝移植至关重要。供体血小板减少需谨慎看待,并被视为捐赠的相对禁忌证。哈里斯血小板综合征(HPLS)是一种最近描述的无症状血小板减少的病因,是遗传性巨血小板疾病的一种亚型,在印度东北部更为常见。在此,我们介绍我们使用HPLS的活体肝供体的经验。我们回顾性收集了所有接受供体肝切除术的HPLS患者的数据。5例患者被诊断为患有HPLS。术中平均失血量为350毫升。术后第1天,所有患者的乳酸水平均<2毫摩尔/升。所有患者均无肝切除术后肝衰竭的任何特征,国际标准化比值在术后第5天恢复正常。需要认识到HPLS的良性性质。这将避免不必要地排除那些否则会提供合适移植物的合适候选者。

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