Abstract:
IgD multiple myeloma is uncommon. Patients generally present at a younger age and have shorter progression free and overall survivals (OSs). Its rarity has inhibited development of a specific risk stratification system or informed best treatment protocols. We present interphase fluorescence in situ hybridization results from a group of 29 cases. These showed evidence of a decreased male to female ratio, decreased OS in patients aged 70 and over, better outcomes in those with kappa light chain restriction, and CD56 positive patients had longer survivals than those lacking CD56. We discuss the biology of IgD multiple myeloma, the need for prospective studies, and challenges for improvements in diagnosis and treatment. We suggest an International Register to accelerate development of best practice guidelines for diagnosis, risk stratification, and treatment.
摘要:
IgD多发性骨髓瘤并不常见。患者通常年龄较小,无进展和总体生存期(OS)较短。它的稀有性抑制了特定风险分层系统或最佳治疗方案的发展。我们介绍了29例病例的间期荧光原位杂交结果。这些证据表明男女比例下降,70岁及以上患者的OS降低,那些有κ轻链限制的人有更好的结果,CD56阳性患者的生存时间比缺乏CD56的患者长。我们讨论IgD多发性骨髓瘤的生物学,需要前瞻性研究,以及改进诊断和治疗的挑战。我们建议建立国际注册,以加快制定诊断最佳实践指南,风险分层,和治疗。