Neurologic aspects of plasma cell disorders.
Neurologic aspects of plasma cell disorders.
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DOI：
10.1016/b978-0-7020-4087-0.00073-5
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发表时间：
2014-01-01
影响因子：
--
通讯作者：
Stiff, Patrick
中科院分区：
文献类型：
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作者：
Sobol, Urszula;Stiff, Patrick
浆细胞病症构成以浆细胞的异常克隆的出现为特征的广谱疾病，其通常表现为单克隆免疫球蛋白的产生（单克隆丙种球蛋白病）。浆细胞的过度产生和随后的单克隆丙种球蛋白病可能是恶性的或癌前病变。意义不明的单克隆丙种球蛋白病（MGUS）是一个良性过程具有恶性潜力的例子。多发性骨髓瘤、瓦尔登斯特伦巨球蛋白血症、POEMS（多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤变化）综合征和AL淀粉样变性（免疫球蛋白轻链淀粉样变性）是需要治疗的恶性浆细胞疾病的实例。潜在浆细胞疾病的神经系统表现可能是可变的，通常具有治疗挑战性。它们的范围可以从治疗疾病的治疗引起的轻度症状到与疾病本身相关的临床显著和危及生命的并发症。周围神经系统比中枢神经系统更常见。周围神经病变是一种常见表现，与所有浆细胞疾病（MGUS、多发性骨髓瘤、POEMS综合征、瓦尔登斯特伦巨球蛋白血症和AL淀粉样变性）相关，不同组之间的体征和症状存在显著差异。中枢神经系统表现的实例包括脊髓病理学，例如来自椎骨塌陷或浆细胞瘤的脊髓压迫。颅内受累罕见，但可发生于脑实质浸润、软脑膜受累和肿瘤样病变，如AL淀粉样变性中的淀粉样瘤和多发性骨髓瘤中的浆细胞瘤。脑病可由于与基础浆细胞疾病相关的代谢紊乱而发生，包括多发性骨髓瘤中的高钙血症和尿毒症以及Waldenstrom巨球蛋白血症中的高粘滞性。本章详细讨论了各种浆细胞疾病及其神经系统表现。
Plasma cell disorders make up a broad spectrum of diseases that are characterized by the appearance of an abnormal clone of plasma cells, which typically manifests as a production of monoclonal immunoglobulin protein (monoclonal gammopathy). The overproduction of plasma cells and subsequent monoclonal gammopathy may be malignant or a premalignant process. Monoclonal gammopathy of undetermined significance (MGUS) is an example of a benign process with a malignant potential. Multiple myeloma, Waldenstrom macroglobulinemia, POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome, and AL amyloidosis (immunoglobulin light chain amyloidosis) are examples of malignant plasma cell disorders which require treatment. Neurologic manifestations of an underlying plasma cell disorder can be variable and typically challenging to treat. They can range from mild symptoms resulting from therapy to treat the disorder to clinically significant and life-threatening complications related to the disease itself. The peripheral nervous system is more commonly affected than the central nervous system. Peripheral neuropathy is a frequent manifestation and is associated with all of the plasma cell disorders (MGUS, multiple myeloma, POEMS syndrome, Waldenstrom macroglobulinemia and AL amyloidosis) with notable differences in the signs and symptoms among the different groups. Examples of central nervous system manifestations include spinal cord pathology, such as spinal cord compression from vertebral collapse or plasmacytoma. Intracranial involvement is rare but can occur from brain parenchyma infiltration, leptomeningeal involvement, and tumor-like lesions, such as amyloidoma in AL amyloidosis and plasmacytoma in multiple myeloma. Encephalopathy can occur due to metabolic disturbances related to the underlying plasma cell disorder, including hypercalcemia and uremia in multiple myeloma and hyperviscosity in Waldenstrom macroglobulinemia. Included in this chapter is a detailed discussion of the various plasma cell disorders and their spectrum of neurologic manifestations.