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以冷凝集素综合征为首发表现的LPL/WM一例

             

摘要

Lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia (LPL/WM)is a rare non-Hodgkin lymphoma,characterized as elevated levels of monoclonal IgM induced by lymphoplasmacytosis, constantly involvement with bone marrow,lymph node and spleen. LPL/WM have a low incidence and is diffi-cult to diagnose. In this article,we reported 1 case of typical LPL/WMwith cold agglutinin syndrome. The pa-tient received bone marrow biopsy,immunofixation electrophoresis and flow cytometry to confirm the diagnosis. He only received TD (thalidomide and dexamethasone)due to alleviated symptoms. After corresponding treat-ment,the patient was alleviated and his condition was stable during subsequent follow-up. For subjects suspec-ted with LPL/WM,bone marrow biopsy,immunofixation electrophoresis and flow cytometry should be per-formed for early diagnosis. After the diagnosis is confirmed,individualized therapy should be delivered accord-ing to the patients' conditions.%淋巴浆细胞淋巴瘤/华氏巨球蛋白血症(LPL/WM)是少见的非霍奇金淋巴瘤,以淋巴浆细胞增生引起血清单克隆IgM增高为特点,通常累及骨髓、淋巴结、脾脏,其发病率较低,不易诊断。该文报道1例以冷凝集素综合征为首发表现的LPL/WM,行骨髓活组织病理学检查(活检)、血清免疫固定电泳、流式细胞学检验明确诊断,确诊后仅接受TD (沙利度胺、地塞米松)方案治疗。经治疗后患者病情好转,随访情况稳定。临床上对于疑为LPL/WM的患者,应尽快行骨髓活检、血清免疫固定电泳、流式细胞学检验等以尽早确诊,确诊后应根据患者病情选择适合的个体化治疗方案。

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