浆细胞白血病的临床特征及诊治进展

Clinical characteristics of plasma cell leukemia and progress in its diagnosis and treatment

浆细胞白血病(PCL)是一种侵袭性强、进展快、预后差的罕见恶性浆细胞肿瘤。PCL的年发病率为(0.04~0.05)/10万。依据有无多发性骨髓瘤(MM)病史,可将PCL分为原发性浆细胞白血病(PPCL)和继发性浆细胞白血病(SPCL),PPCL约占PCL的60%,发病时即处于白血病阶段,既往无MM病史;SPCL占40%,多为MM的一种终末期表现,也可继发于华氏巨球蛋白血症、B细胞淋巴瘤、慢性淋巴细胞白血病和淀粉样变等,由MM进展为SPCL的患者占全部MM患者的2%~4%。由于PCL发病率低、临床异质性强,其循证学依据相对缺乏,文章就PCL的临床特征及相关诊治进展进行综述。

Plasma cell leukemia(PCL)is a plasma cell proliferative disorder with strong invasiveness,rapid progression and poor prognosis.The incidence of PCL is about(0.04-0.05)/100000 per year.According to the multiple myeloma(MM)history,PCL can be divided into primary plasma cell leukemia(PPCL)and secondary plasma cell leukemia(SPCL).PPCL accounts for about 60%of PCL,and it is in the stage of leukemia at diagnosis and has no history of MM.SPCL accounts for the remaining 40%of PCL,and mostly shows as the MM end-stage manifestation,but also can be secondary to Waldenstrom macroglobulinemia,B-cell lymphoma,chronic lymphoblastic leukemia,amyloidosis,etc.Patients who progress from MM to SPCL account for 2%-4%of all MM patients.Due to the low incidence and strong clinical heterogeneity of PCL,the evidence-based medicine about PCL is relatively lacking,this article reviews the clinical characteristics of PCL and progress in its diagnosis and treatment.