非IgM型淋巴浆细胞淋巴瘤临床及生物学特征研究

Clinical and biological characteristics of non-IgM lymphoplasmacytic iymphoma

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）,slgM阳性1例,CD23和CDllc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.

Objective To observe the clinical and biological characteristics of Non-IgM-secreting lymphoplasmacytic lymphoma （LPL） and draw the differences between non-IgM LPL and Waldenstr（o）m 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 CDllc 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）.Conclusions The clinical and biological characteristics had no significantly difference between non-IgM LPL and WM.