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实体器官移植受者中的淀粉样变性诊断。

Amyloidosis Diagnosed in Solid Organ Transplant Recipients.

机构信息

Centre for Amyloidosis & Acute Phase Proteins, Division of Medicine (Royal Free Campus), London, UK.

出版信息

Transplantation. 2020 Feb;104(2):415-420. doi: 10.1097/TP.0000000000002813.

Abstract

BACKGROUND

Development of amyloidosis post solid-organ transplantation has not been reported, although plasma cell neoplasms are a rare form of posttransplant lymphoproliferative disorder, which could be complicated by light chain amyloidosis (AL) amyloidosis.

METHODS

We searched our database of 5112 patients seen between 1994 and 2018 with a diagnosis of amyloidosis post solid-organ transplant. Patients were excluded if the amyloid diagnosis preceded the transplant date. The indication and type of organ transplant were recorded in addition to the amyloidosis type, organs involved, treatment given, and survival.

RESULTS

Thirty patients were identified. The median age at diagnosis with amyloidosis was 52 years (range 33-77). The median time from transplantation to diagnosis was 10.5 years (0.58-36). The grafts were kidney (N = 25, 83.3%), liver (N = 2, 6.7%), heart (N = 2, 6.7%), and combined heart, lung, and kidney (N = 1, 3.3%). The type of amyloidosis was systemic AL (N = 14, 47%), serum amyloid A amyloidosis (AA) (N = 11, 37%), localized AL (N = 3, 10%), wild-type transthyretin amyloidosis (ATTR) (N = 1, 3.3%), and amyloid of uncertain type (N = 1, 3.3%). Renal graft dysfunction was seen in 11 of 25 (44%) cases. Median graft survival was 185 months (96-269), and median survival from diagnosis with amyloidosis was 45 months (2-89); median survival by amyloidosis type was localized AL: 64 months (20-67), systemic AL: 23.5 months (0-95), ATTR amyloidosis: 17 months, and AA, 15 months (0-77).

CONCLUSIONS

This series is the first description of amyloidosis post solid-organ transplant; 30 cases among 5112 amyloid patients >24 years suggests that amyloidosis may occur post solid-organ transplantation with an overall poor survival.

摘要

背景

实体器官移植后发生淀粉样变性尚未见报道,尽管浆细胞肿瘤是移植后淋巴增殖性疾病的一种罕见形式,可能并发轻链淀粉样变性(AL)淀粉样变性。

方法

我们检索了 1994 年至 2018 年间数据库中 5112 例确诊为实体器官移植后淀粉样变性的患者。如果淀粉样变性的诊断先于移植日期,则排除这些患者。记录了淀粉样变性的类型、受累器官、给予的治疗和生存情况。

结果

共确定了 30 例患者。淀粉样变性诊断时的中位年龄为 52 岁(范围 33-77 岁)。从移植到诊断的中位时间为 10.5 年(0.58-36 年)。移植物为肾脏(N=25,83.3%)、肝脏(N=2,6.7%)、心脏(N=2,6.7%)和心脏、肺和肾脏联合(N=1,3.3%)。淀粉样变性的类型为系统性 AL(N=14,47%)、血清淀粉样蛋白 A 淀粉样变性(AA)(N=11,37%)、局限性 AL(N=3,10%)、野生型转甲状腺素蛋白淀粉样变性(ATTR)(N=1,3.3%)和不确定类型的淀粉样变性(N=1,3.3%)。25 例中有 11 例(44%)出现肾脏移植物功能障碍。中位移植物存活时间为 185 个月(96-269 个月),淀粉样变性诊断后的中位生存时间为 45 个月(2-89 个月);按淀粉样变性类型的中位生存时间为局限性 AL:64 个月(20-67 个月)、系统性 AL:23.5 个月(0-95 个月)、ATTR 淀粉样变性:17 个月和 AA:15 个月(0-77 个月)。

结论

本系列是实体器官移植后淀粉样变性的首次描述;5112 例>24 岁的淀粉样变性患者中有 30 例提示淀粉样变性可能发生在实体器官移植后,整体预后较差。

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