Fend F
Institut für Pathologie, Liebermeisterstr. 8, 72074 Tübingen.
Pathologe. 2010 Oct;31 Suppl 2:188-92. doi: 10.1007/s00292-010-1375-1.
Plasma cell myeloma (PCM) and related immunosecretory disorders are a group of B-cell proliferations with a wide clinical and prognostic spectrum, characterized by the production of monoclonal immunoglobulin by immortalized plasma cells. Recent years have seen an explosion in knowledge on the genetic basis and biology of these diseases, followed by improved clinical risk stratification and the introduction of novel therapeutic concepts, such as treatment with proteasome inhibitors or immunomodulatory substances. PCM is a common malignancy, accounting for approximately 10% of all hematological neoplasms. There is good evidence to support a multistep transformation process in plasma cell neoplasms, which corresponds to clinically discernible disease stages. Monoclonal gammopathy of unknown significance is a common asymptomatic precursor lesion for PCM which carries an approximately 1% annual risk for progression. Terminal disease stages are characterized by increasing genetic complexity and independence from bone marrow stromal cells and show a rapidly increasing tumour load with severe clinical symptoms. Modern diagnostics of plasma cell neoplasms require inclusion of clinical, morphological, immunophenotypical and cytogenetic features to allow for individual risk assessment and therapy planning.
浆细胞骨髓瘤（PCM）及相关免疫分泌性疾病是一组B细胞增殖性疾病，具有广泛的临床和预后范围，其特征是永生浆细胞产生单克隆免疫球蛋白。近年来，人们对这些疾病的遗传基础和生物学的认识有了飞速发展，随后临床风险分层得到改善，并且引入了新的治疗理念，如使用蛋白酶体抑制剂或免疫调节物质进行治疗。PCM是一种常见的恶性肿瘤，约占所有血液系统肿瘤的10%。有充分证据支持浆细胞肿瘤的多步骤转化过程，这与临床上可辨别的疾病阶段相对应。意义未明的单克隆丙种球蛋白病是PCM常见的无症状前驱病变，每年进展风险约为1%。终末期疾病阶段的特征是遗传复杂性增加以及对骨髓基质细胞的依赖性降低，并显示肿瘤负荷迅速增加且伴有严重临床症状。浆细胞肿瘤的现代诊断需要纳入临床、形态学、免疫表型和细胞遗传学特征，以便进行个体风险评估和治疗规划。
