预防性血浆置换联合利妥昔单抗序贯治疗华氏巨球蛋白血症

目的：探讨华氏巨球蛋白血症（WM）的临床特征，提高利妥昔单抗治疗WM的认识。方法：报告1例采用预防性血浆置换后给予利妥昔单抗序贯治疗血清IgM〉50g／L的WM并结合文献进行复习。结果：患者为老年男性。无淋巴结和肝脾肿大，主要表现为血清IgM明显增高（IgM631g／L）、伴有高黏滞血症表现、贫血，骨髓示淋巴细胞弥漫性浸润，免疫分型符合WM。经预防性血浆置换后给予利妥昔单抗为基础的方案治疗，出现轻微IgM反跳，未发生高黏滞血症加重和其他并发症。接受治疗5月后获得主要反应（IgM下降〉50％）、23月后接近非常好的部分缓解水平（IgM下降／〉90％）。结论：WM属于慢性B淋巴细胞增殖性肿瘤，临床少见，多发生于老年，有症状者需接受治疗。接受以利妥昔单抗为基础的治疗后，可发生IgM反跳，严重时可使病情加重，治疗前IgM〉50g／L者尤为明显，IgM反跳并不意味利妥昔单抗治疗失败，预防性血浆置换对降低反跳发生及IgM增高的程度有积极意义。

Rituximab therapy following prophylactic plasmapheresis in the treatment of Waldenstrom macroglobulinemia

Objective：To explore the clinical features of Waldenstrom macroglobalinemia（WM） and improve the recognition of rituximab - based regimen in the treatment of WM. Methods： One WM patient with serum IgM level of more than 50g/L receiving rituximab therapy following prophylactic plasmapheresis was reported and the related articles were reviewed. Results： An elder male patient showed no lymphadenopathy and hepatosplenomegaly, mainly presenting with marked increasing in sermn IgM level（IgM 63.1 g/L） which lead to the symptoms of hyperviseosity,anemia and diffuse proliferation of lymphoeytes in the marrow. Lymphocytes showed abnormal immunophenotypes which were compatible with WM. The patient received rituximab therapy following prophylactic plasmapheresis and experienced a slight rise in s ermn IgM （IgM flare ） at 3 months afler initial therapy and had no evidence of worsening symptoms of hyperviseosity or creating complications. Then, he obtained major response （ 〉 50% reduction of serum monoclonal igM level） after 5 months. With a follow- up of 23 months,the patient approached very good partial remission （ ≥90% reduction of serum monoelonal IgM level）. Conclusion：WM is a rare B - cell lymphoproliferative disorder and generally affects elderly patients. The patients with symptomatic WM should be treated. Paradoxical spikes in serum IgM levels may occur in WM patients treated with rituximab and can result in worsening symptoms attributed to increasing serum viscosity,particularly in patients with a pretherapy igM levels of 〉 50g/L. Rituximab - related igM flare is not indicative of treatment failure. Prophylactic plasmapheresis is an active means to lower the incidence of igM flare or reduce the extent of increasing sermn IgM level.