Rajkumar S Vincent, Dispenzieri Angela, Kyle Robert A
Division of Hematology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
Mayo Clin Proc. 2006 May;81(5):693-703. doi: 10.4065/81.5.693.
The spectrum of plasma cell disorders is broad. Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma are asymptomatic disorders characterized by monoclonal plasma cell proliferation in the bone marrow in the absence of end-organ damage. Waldenström macroglobulinemia typically involves an ontogenically less mature lymphoplasmacytic bone marrow cell and is characterized by secretion of a monoclonal IgM protein. Solitary plasmacytoma is the only known potentially curable plasma cell disorder. Finally, AL (immunoglobulin light chain) amyloidosis and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome are disorders characterized by low tumor burden but profound multisystemic disease. Updated diagnostic criteria for these disorders, risk stratification models to determine prognosis, and the current management of these diverse entitles are discussed in this review.
浆细胞疾病的范围很广。意义未明的单克隆丙种球蛋白病和冒烟型多发性骨髓瘤是无症状性疾病，其特征是骨髓中存在单克隆浆细胞增殖且无终末器官损害。华氏巨球蛋白血症通常累及发育上不太成熟的淋巴浆细胞骨髓细胞，其特征是分泌单克隆IgM蛋白。孤立性浆细胞瘤是唯一已知的潜在可治愈的浆细胞疾病。最后，AL（免疫球蛋白轻链）淀粉样变性和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改变）综合征是肿瘤负荷低但有多系统严重疾病的疾病。本文综述了这些疾病的最新诊断标准、用于确定预后的风险分层模型以及对这些不同疾病的当前管理方法。
