AL淀粉样变性（AL-A）的患者如果同时病变累及心脏和肾脏则其预后不佳。我们报道了首例因为系统性AL淀粉样变性而行心肾联合移植的长期存活患者，该患者未行自体干细胞移植。该受者是一名46岁的老年男性，终末期肾脏功能衰竭，AL-A严重累及心脏。移植前，两个疗程的美法兰和强的松诱导出现了血象上部分缓解，表现为循环游离轻链水平下降但心脏和肾脏功能未有改善。患者进行心肾联合移植进行救治。在随访期（36个月）间，浆细胞恶性增生获得完全缓解，而游离lambda轻链水平正常，心脏和肾脏移植物未再发生淀粉样物质沉积。该病例报道显示不合并干细胞移植的心肾联合移植是AL-A患者发生严重器官功能衰竭和血象病变后的另一种治疗选择。

Audard V, Matignon M, Weiss L, Remy P, Pardon A, Haioun C, Belhadj K, Salomon L, Hillon ML, Sahali D, Vermes E, Lang P, Grimbert P.
APHP, Hôpital Henri Mondor, Department of Nephrology and Renal Transplantation; Institut Francilien de recherche en Néphrologie et Transplantation (IFRNT), Univ Paris 12, Créteil, 94010, France.

Simultaneous cardiac and renal involvement is associated with a particularly poor prognosis in patients with AL amyloidosis (AL-A). We report the first case of a successful long-term outcome of combined heart and kidney transplantation not followed by autologous stem cell transplantation in a patient with systemic AL-A. The recipient was a 46-year-old man with end-stage renal failure associated with serious cardiac involvement in the context of AL-A. Before transplantation, two courses of oral melphalan plus prednisone induced partial hematologic remission, as shown by the decrease in circulating free light chain with no improvement of renal or heart function. The patient underwent combined heart and kidney transplantation as a rescue treatment. During the follow-up period (36 months), plasma cell dyscrasia remains in complete remission, with normal free lambda light chain levels and no recurrence of amyloid deposition on heart and kidney grafts. This case report demonstrates that combined heart and kidney transplantation not systematically associated with stem cell transplantation may be considered an additional therapeutic option in AL-A patients with severe organ dysfunction and partial hematologic remission.