关键词: ibrutinib myasthenia gravis paraneoplastic waldenstrom macroglobulinemia

Mesh : Humans Waldenstrom Macroglobulinemia / drug therapy complications Male Adenine / analogs & derivatives therapeutic use Middle Aged Piperidines / therapeutic use Myasthenia Gravis / drug therapy complications diagnosis Pyrimidines / therapeutic use Pyrazoles / therapeutic use Paraneoplastic Syndromes, Nervous System / drug therapy etiology Treatment Outcome

来  源:   DOI:

Abstract:
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.
摘要:
副肿瘤综合征是由肿瘤引起的广泛的体征和症状,归因于肿瘤细胞产生的物质,或回应它。重症肌无力(MG)是一种众所周知的副肿瘤神经综合征(PNS),经常与胸腺异常有关,但很少在淋巴浆细胞性淋巴瘤患者中报道。这项研究介绍了一名52岁的印度尼西亚男性患者,该患者被诊断为Waldenstrom巨球蛋白血症(WM)。一种罕见的B细胞肿瘤,在出现新的肌无力危象后。Ibrutinib作为癌症靶向治疗,患者的MG特征得到改善.这是第一例报告,介绍了依鲁替尼在WM伴有肌无力危象的治疗反应。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依鲁替尼对该患者的治疗反应。以及总结先前合并MG和WM的病例报告。MG应被视为副肿瘤恶性肿瘤综合征,包括WM,在诊断检查期间。当患者可用时,也应考虑伊布替尼,由于其在先前治疗和未治疗的患者中都有足够的反应。
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