浆细胞白血病与多个髓外浆细胞瘤研究进展

Research progress of plasma cell leukemia and multiple extramedullary plasmacytoma

浆细胞白血病(PCL)和多个髓外浆细胞瘤(MEP)既可原发,亦可由多发性骨髓瘤(MM)继发而来。目前此组疾病的发病率随着MM生存期的延长而渐见增多。与MM相比,PCL侵犯器官更常见,而MEP常累及头部、颈部或脊柱旁区的软组织。高度耐药和早期复发是PCL和MEP预后不良的主要原因。单一烷化剂和常规联合化学治疗对此组疾病基本无效。大剂量化学治疗加干细胞移植治疗的疗效尚未被证实。硼替唑米对PCL和MEP较为有效,无效时可予以来那利度胺为基础的联合治疗。

Plasma cell leukemia （PCL） and multiple extramedullary plasmacytomas （MEP） are very aggres sive malignancies. These can be de-novo or secondary due to progressive prior multiple myeloma （MM）. Recent reports suggest an increase in incidence of these disorders. Compared to MM, organ invasion is common in PCL, while soft tissue tumors involving the head, neck or paraspinal area are common sites for MEP. Patients with PCL and MEP have aggressive disease characterized by highly resistance to therapy and early relapse. The single alkylating agents and conventional combination chemotherapy have not shown activity in these disorders. Even high dose chemotherapy with stem ceU transplant rescue has not been able to demonstrate improvement in survival outcome. Novel anti-plasma cell agents, such as the bortezomib, appear to be active against PCL and MEP. For patients intolerant of bortezomib, lenalidomide-based combination treatment can be a reasonable alternative.