Objective: To observe the clinical and biological characteristics of Non-IgM-secreting lymphoplasmacytic lymphoma (LPL) and draw the differences between non-IgM LPL and Waldenström macroglobulinemia (WM).
Methods: Records of 13 patients with non-IgM LPL were retrospectively analyzed between January 2000 and December 2013. The cytogenetic aberrations were detected by fluorescence in situ hybridisation (FISH).
Results: In the cohort, 7 males and 6 females with a median age of 63 years (range 43 to 74), two patients were IgA secreting, 6 with IgG secreting and 5 patients without monoclonal globulin. The major complaint at diagnosis included anemia associated symptom (53.8%), mucocutaneous hemorrhage and superficial lymphadenopathy (15.4%). Eight patients had B symptom at diagnosis. All of the 13 patients had bone marrow involvement and anemia, and 10 patients had 2 or 3 lineage cytopenia. In 5 patients with available immunophenotypic data, all expressed CD19, CD20, CD22 and CD25, but missed the expression of CD10, CD103 and CD38. Two cases had CD5 or sIgM positive alone. Another 2 patients were CD23 or CD11c positive and 3 patients were FMC7 positive. Cytogenetic aberrations had been detected by FISH in 7 patients, but only two (28.6%) patients had aberrations with del(6q).
Conclusion: The clinical and biological characteristics had no significantly difference between non-IgM LPL and WM.
目的: 探索非IgM型淋巴浆细胞淋巴瘤（LPL）患者的临床及生物学特征，并与华氏巨球蛋白血症（WM）进行比较，探索两者的异同。
方法: 对2000年1月至2013年12月收治的13例非IgM型LPL患者临床资料进行回顾性分析，应用荧光原位杂交技术（FISH）对其中7例患者标本进行检查。
结果: 13例患者中，男7例，女6例，中位发病年龄63(43~74）岁。2例分泌单克隆IgA, 6例分泌单克隆IgG，5例不分泌单克隆性免疫球蛋白。以贫血为主要表现者7例，以皮肤黏膜出血和浅表淋巴结肿大为主要表现者各2例，出现B症状（发热、盗汗、体重减轻）者8例。所有患者均骨髓受累并表现贫血，10例患者为血常规2系或以上减少。行流式细胞术检查的5例患者中CD19、CD20、CD22和CD25均阳性，CD10、CD38和CD103均阴性，CD5弱阳性1例（该患者CD23阴性），sIgM阳性1例，CD23和CD11c阳性各2例，FMC7阳性3例。7例患者行细胞遗传学检查，未见异常核型，应用FISH检查发现其中2例患者伴有6q缺失。
结论: 结合文献报道，非IgM型LPL与WM患者临床及生物学特征相似。