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胸腺瘤病理学与重症肌无力结局。

Thymoma pathology and myasthenia gravis outcomes.

机构信息

Ellen and Martin Prosserman Centre for Neuromuscular Diseases, University Health Network, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.

Department of Laboratory Medicine and Pathology, University Health Network, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.

出版信息

Muscle Nerve. 2021 Jun;63(6):868-873. doi: 10.1002/mus.27220. Epub 2021 Mar 16.

Abstract

INTRODUCTION

There is limited evidence regarding the impact of World Health Organization (WHO) subtype of thymoma on post-thymectomy outcome of thymoma-associated myasthenia gravis (TAMG). The objective was to determine if the pathological subtypes of thymoma were associated with post-thymectomy outcomes of myasthenia gravis (MG), in patients with TAMG.

METHODS

We performed a retrospective study of consecutive patients with TAMG who attended the neuromuscular clinic between January 2018 and December 2019 with a minimum follow-up of 1 y after thymectomy. Outcome measures were MG Impairment Index (MGII), single-simple question (SSQ), Myasthenia Gravis Foundation of America post-intervention status (MGFA PIS) and non-responder MG status at last assessment.

RESULTS

Ninety-five patients were included; mean age at onset was 48.1 ± 12.1 y; 54(56.8%) were females. Thirteen patients developed MG post-thymectomy. The most common thymoma was WHO type B2 in 39 (41.1%). Most patients (40, 42.1%) had Masaoka stage II thymoma. There was no association of thymoma subtypes or Masaoka stage of disease with age, gender, MG phenotype, serology, post-thymectomy onset, interval from onset to thymectomy, MGII, SSQ, MGFA PIS, or non-responder status. Associations were found between positive serology and lower MGII (11.1 ± 14.2 vs 23 ± 12.9, P = .050), thymic follicular hyperplasia (TFH) and higher SSQ (89.3 ± 11.7 vs 80.1 ± 20.2, P-.043), and lack of recurrence and higher SSQ (84.1 ± 18 vs 72.5 ± 20, P = .037).

DISCUSSION

The WHO pathological subtype of thymoma did not correlate with MG outcomes. However, positive acetylcholine antibody serology, presence of TFH, and non-recurrence of thymoma predict a favorable outcome.

摘要

简介

关于世界卫生组织(WHO)胸腺瘤亚型对胸腺瘤相关重症肌无力(TAMG)胸腺切除术术后结果的影响,证据有限。目的是确定 TAMG 患者的胸腺瘤病理亚型是否与重症肌无力(MG)的胸腺切除术术后结果相关。

方法

我们对 2018 年 1 月至 2019 年 12 月期间在神经肌肉诊所就诊的 TAMG 连续患者进行了回顾性研究,这些患者在胸腺切除术后至少随访 1 年。结果测量指标为肌无力指数(MGII)、单一简单问题(SSQ)、美国重症肌无力基金会干预后状态(MGFA PIS)和最后评估时非应答 MG 状态。

结果

共纳入 95 例患者,发病时平均年龄为 48.1±12.1 岁,54 例(56.8%)为女性。13 例患者在胸腺切除术后出现 MG。最常见的胸腺瘤为 WHO 型 B2 型,共 39 例(41.1%)。大多数患者(40 例,42.1%)的胸腺瘤分期为 Masaoka Ⅱ期。胸腺瘤亚型或疾病的 Masaoka 分期与年龄、性别、MG 表型、血清学、胸腺切除术后发病、发病至胸腺切除的时间间隔、MGII、SSQ、MGFA PIS 或非应答状态无相关性。阳性血清学与较低的 MGII(11.1±14.2 与 23±12.9,P=0.050)、胸腺瘤滤泡增生(TFH)与较高的 SSQ(89.3±11.7 与 80.1±20.2,P=0.043)和无复发与较高的 SSQ(84.1±18 与 72.5±20,P=0.037)之间存在相关性。

讨论

胸腺瘤的 WHO 病理亚型与 MG 结果无关。然而,乙酰胆碱抗体阳性血清学、TFH 存在和胸腺瘤无复发预测预后良好。

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