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胸腺瘤病理和重症肌无力(MG)

已有 120 次阅读2021-4-5 20:07 |个人分类:TET学习|系统分类:医学科学| 胸腺瘤, 重症肌无力

胸腺瘤病理和重症肌无力。Thymoma pathology and myasthenia gravis outcomes
概述: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)胸腺切除术后结局的影响的证据有限。目的是确定在TAMG患者中,胸腺瘤的病理亚型是否与重症肌无力(MG)的胸腺切除术后预后相关。
方法:我们对2018年1月至2019年12月间在神经肌肉诊所就诊的TAMG连续患者进行了回顾性研究,在胸腺切除术后至少随访了1年。结果指标为MG障碍指数(MGII),单因素问题(SSQ),美国重症肌无力基金会干预后状态(MGFA PIS)和无反应的MG状态(最后评估)。
结果:纳入95例患者。平均发病年龄为48.1±12.1 y; 54名(56.8%)是女性。 13名患者发生了胸腺切除术后的MG。最常见的胸腺瘤类型的WHO B2型(39,41.1%)。大多数患者(40,42.1%)患有Masaoka II期胸腺瘤。胸腺瘤亚型或疾病的Masaoka分期与年龄,性别,MG表型,血清学,胸腺切除术后发作,发作至胸腺切除后时间间隔,MGII,SSQ,MGFA PIS或无反应者状态无关联。发现血清学阳性与MGII较低(11.1±14.2 vs 23±12.9,P = .050),胸腺滤泡增生(TFH)和SSQ较高(89.3±11.7 vs 80.1±20.2,P-.043)之间存在关联复发率和较高的SSQ(84.1±18 vs 72.5±20,P = .037)。
讨论:胸腺瘤的WHO病理亚型与MG结果无关。然而,阳性的乙酰胆碱抗体血清学检查,TFH的存在和胸腺瘤的复发均预示良好的预后。
关键词:重症肌无力;结果;分期胸腺切除术胸腺瘤亚型。
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.
Keywords: myasthenia gravis; outcomes; staging; thymectomy; thymoma subtype.

Menon D, Katzberg H, Barnett C, Pal P, Bezjak A, Keshavjee S, Bril V. Thymoma pathology and myasthenia gravis outcomes. Muscle Nerve. 2021 Mar 6. doi: 10.1002/mus.27220. Epub ahead of print. PMID: 33675078.

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