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意义未明的单克隆丙种球蛋白病的神经系统表现。

Neurological manifestations of MGUS.

作者信息

Cibeira M Teresa, Rodríguez-Lobato Luis Gerardo, Alejaldre Aida, Fernández de Larrea Carlos

机构信息

Hematology Department, Amyloid and Myeloma Unit, Hospital Clínic de Barcelona and Institut d´Investigacions Biomèdiques August Pi I Sunyer, University of Barcelona, Barcelona, Spain.

Neurology Department, Amyloid and Myeloma Unit, Hospital Clínic de Barcelona and Institut d´Investigacions Biomèdiques August Pi I Sunyer, University of Barcelona, Barcelona, Spain.

出版信息

Hematology Am Soc Hematol Educ Program. 2024 Dec 6;2024(1):499-504. doi: 10.1182/hematology.2024000665.

Abstract

Monoclonal gammopathy of undetermined significance (MGUS) is a highly prevalent disorder characterized by a small bone marrow plasma cell or lymphoplasmacytic clone (less than 10%) that produces a small amount of monoclonal paraprotein without associated organ damage. Most patients with MGUS display benign behavior indefinitely, but some progress to an overt malignancy, and others develop organ damage despite no increase in monoclonal protein, resulting in the so-called MG of clinical significance (MGCS). This concept includes different disorders depending on the organ involved, and among them, MG of neurological significance (MGNS) constitutes a real challenge from both a diagnostic and therapeutic point of view. Diagnosis is particularly difficult due to MGNS's heterogeneous clinical presentation and common lack of a diagnostic biopsy. On the other hand, the complexity of treatment lies in the lack of standardized regimens and the common irreversibility of neurological damage. Focusing on the neurological manifestations of MGUS affecting the peripheral nervous system, we describe 3 illustrative cases from daily practice and discuss different aspects of diagnosis to treatment, emphasizing the need for multidisciplinary management based on the close collaboration of neurologists and hematologists.

摘要

意义未明的单克隆丙种球蛋白病(MGUS)是一种高度常见的疾病,其特征是骨髓中有一个小的浆细胞或淋巴浆细胞克隆(少于10%),产生少量单克隆副蛋白,且无相关器官损害。大多数MGUS患者长期表现为良性,但有些会进展为明显的恶性肿瘤,还有些患者尽管单克隆蛋白没有增加,但仍出现器官损害,导致所谓的具有临床意义的MG(MGCS)。这一概念包括根据受累器官不同的多种疾病,其中,具有神经学意义的MG(MGNS)从诊断和治疗角度来看都是一项真正的挑战。由于MGNS临床表现异质性且通常缺乏诊断性活检,诊断尤为困难。另一方面,治疗的复杂性在于缺乏标准化方案以及神经损伤通常不可逆转。聚焦于影响周围神经系统的MGUS的神经学表现,我们描述了3例日常临床病例,并讨论了从诊断到治疗的不同方面,强调了基于神经科医生和血液科医生密切合作的多学科管理的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f3df/11665721/46bdf4909111/hem.2024000665_s1.jpg

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