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序贯肝、肾和自体干细胞移植治疗 AL 淀粉样变性:一例报告。

Sequential Liver, Kidney, and Autologous Stem Cell Transplant for AL Amyloidosis: A Case Report.

机构信息

Department of General Surgery and Organ Transplantation, Yeni Yuzyil University, School of Medicine, Private Gaziosmanpasa Hospital, Istanbul, Turkey.

Department of Interventional Radiology, Yeni Yuzyil University, School of Medicine, Private Gaziosmanpasa Hospital, Istanbul, Turkey.

出版信息

Transplant Proc. 2022 Jan-Feb;54(1):197-201. doi: 10.1016/j.transproceed.2021.11.009. Epub 2021 Dec 23.

Abstract

AL type amyloidosis is a systemic disease characterized by the accumulation of amyloid fibrils that can affect many organs such as the skin, gastrointestinal tract, heart, lungs, liver, and kidney. The most frequently involved organ in amyloidosis is the kidney, but cardiac amyloidosis with the poor prognosis is amyloid organ involvement. In this study, we present the treatment of a 40-year-old female patient with acute Budd-Chiari syndrome and very severe proteinuria with sequential liver, kidney, and autologous stem cell transplant after the diagnosis of systemic amyloidosis. To reduce the effects of massive proteinuria and very severe hypoalbuminemia, bilateral renal artery embolization was performed first. After the evaluation of the patient, she underwent liver transplant from a deceased donor, and then kidney transplant was performed from her son 1 month later. Afterward, the patient was discharged without any problems and underwent chemotherapy and stem cell transplant for primary AL amyloidosis. She was followed up without any problem in terms of liver, kidney, and stem cell at the 24th postoperative month. This case shows that autologous stem cell transplant after kidney and liver transplant may be a good treatment option in a selected patient with stem cell involvement diagnosed as having AL amyloidosis.

摘要

AL 型淀粉样变病是一种全身性疾病,其特征是淀粉样纤维的积累,可影响皮肤、胃肠道、心脏、肺、肝和肾等许多器官。淀粉样变病最常累及的器官是肾脏,但预后不良的心脏淀粉样变病是淀粉样器官受累。在本研究中,我们介绍了一位 40 岁女性患者的治疗方法,该患者被诊断为系统性淀粉样变病后,先后进行了肝、肾和自体干细胞移植,以治疗急性 Budd-Chiari 综合征和严重的蛋白尿。为了减少大量蛋白尿和严重低白蛋白血症的影响,首先进行了双侧肾动脉栓塞。在对患者进行评估后,她接受了来自已故供体的肝移植,然后在 1 个月后接受了来自她儿子的肾移植。此后,患者无任何问题出院,并接受了原发性 AL 淀粉样变性的化疗和干细胞移植。在术后第 24 个月,患者在肝、肾和干细胞方面无任何问题进行了随访。本病例表明,在诊断为 AL 淀粉样变病并伴有干细胞受累的选定患者中,干细胞移植后进行自体干细胞移植可能是一种很好的治疗选择。

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