Division of Hematology and Medical Oncology, Department of Internal Medicine, Faculty of Medicine Universitas Indonesia - Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia..
Acta Med Indones. 2024 Apr;56(2):233-239.
Paraneoplastic syndrome is a broad spectrum of signs and symptoms due to neoplasm, attributed to substances produced by tumor cells, or in response to it. Myasthenia gravis (MG) is a well-known paraneoplastic neurological syndrome (PNS), frequently associated with thymic abnormalities, but rarely reported in patients with lymphoplasmacytic lymphoma.This study presents the case of a 52-year-old Indonesian male patient who was diagnosed with Waldenstrom macroglobulinemia (WM), a rare B-cell neoplasm, after developing a new onset of MG with myasthenic crisis. the patient's MG features improved with Ibrutinib as a treatment targeted toward cancer. This is the first case report presenting the treatment response of Ibrutinib in WM with myasthenic crisis. The literature was reviewed to explain the possibility of MG as a paraneoplastic syndrome of WM and the treatment response of Ibrutinib for this patient, as well as summarizing previous case reports of concomitant MG and WM.MG should be considered a paraneoplastic malignancy syndrome, including WM, during diagnostic workup. Ibrutinib should also be considered when available to patients, due to its adequate response in both previously treated and treatment naïve patients.
副肿瘤综合征是一种由于肿瘤引起的广泛的症状和体征，归因于肿瘤细胞产生的物质，或对其的反应。重症肌无力（MG）是一种众所周知的副肿瘤神经系统综合征（PNS），常与胸腺异常有关，但在淋巴浆细胞淋巴瘤患者中很少报道。本研究报告了一例 52 岁印度尼西亚男性患者的病例，该患者在发生新的 MG 伴肌无力危象后被诊断为瓦尔登斯特伦巨球蛋白血症（WM），一种罕见的 B 细胞肿瘤。该患者的 MG 特征随着伊布替尼的治疗而改善，伊布替尼是一种针对癌症的靶向治疗药物。这是首例报告伊布替尼治疗伴有肌无力危象的 WM 的病例。本文回顾了文献，以解释 MG 作为 WM 的副肿瘤综合征的可能性，以及伊布替尼对该患者的治疗反应，并总结了以前同时伴有 MG 和 WM 的病例报告。MG 应被视为包括 WM 在内的副肿瘤恶性肿瘤综合征，在诊断性检查期间应考虑到这一点。伊布替尼也应该在有条件的情况下考虑用于患者，因为它在以前治疗过和未治疗过的患者中都有足够的反应。
