Nikhil Patel, Samer Al Hadidi, Sarvari Yellapragada
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引用次数: 0
摘要
沃登斯特罗姆巨球蛋白血症(WM)或淋巴浆细胞性淋巴瘤是一种 B 细胞恶性肿瘤,其特征是骨髓中的淋巴浆细胞分泌大量免疫球蛋白 (Ig)M。此外,恶性细胞还可浸润中枢神经系统,导致多种神经系统并发症,也称为宾-尼尔综合征(Bing Neel Syndrome)。由于 WM 和这些并发症的病理生理学非常独特,其诊断工作和治疗方案也大相径庭。鉴于这种疾病及其并发症的罕见性,几乎没有关于这些并发症治疗的随机对照试验,因此,建议的治疗方案通常基于观察研究。在本病例系列中,我们将介绍三种 WM 病例,每种病例都有其独特的并发症,并讨论病理生理学以及每种并发症目前和未来的治疗方案。
Pathophysiology and Treatments of Complications of Waldenström's Macroglobulinemia.
Waldenstrom's macroglobulinemia (WM) or lymphoplasmacytic lymphoma is a B-cell malignancy characterized by lymphoplasmacytic cells in the bone marrow that secrete high amounts of immunoglobulin (Ig) M. The large pentameric structure of IgM leads to a variety of unique complications in WM, such as hyperviscosity syndrome, cryoglobulinemia and sensory neuropathy. Furthermore, malignant cells can infiltrate the central nervous system and lead to a variety of neurological complications, also known as Bing Neel Syndrome. Because of the unique pathophysiology of WM and these complications, their diagnostic work up and treatment regimens vary greatly. Given the rarity of the disease and their complications, there are little to no randomized controlled trials regarding treatments of these complications and, therefore, suggested treatment regimens are usually based on observational studies. In this case series, we will present three cases of WM, each with their own unique complication, and discuss the pathophysiology along with current and future treatment options for each of the complications presented.