累及骨髓和外周血的成熟小B细胞淋巴瘤7种亚型的诊断分析

Diagnostic analysis of seven subtypes of mature small B-cell lymphoma involving the bone marrow and peripheral blood

目的:探讨慢性累及骨髓和外周血的成熟小B细胞淋巴瘤的诊断及其鉴别诊断方法。方法:回顾昆山市第三人民医院2015年2月至2021年6月诊治的7种类型临床达Ⅳ期的成熟小B细胞淋巴瘤患者27例的临床资料,分析不同检测方法在疾病诊断中的应用价值。结果:大部分患者外周血主要以淋巴细胞比值或绝对值增高为多见;细胞形态学上主要以小至中等体积的成熟淋巴细胞为主,少数骨髓涂片或外周血涂片可见特征性细胞形态改变;流式细胞学检测结果显示表达CD5患者15例,其中慢性淋巴细胞白血病(CLL)11例,套细胞淋巴瘤(MCL)1例,其余3例中有1例为脾弥漫红髓小B细胞淋巴瘤(SDRPL)、2例被列为不能分类型(B-CLPD-u)。CD5阴性患者12例,分别为华氏巨球蛋白血症(WM)8例,脾边缘区淋巴瘤(SMZL)3例,以及滤泡性淋巴瘤(FL)1例。其中2例CD5-CD10阳性患者中,1例确诊为FL,1例为WM。1例SDRPL患者除了表达泛B细胞标志外,同时伴CD5、CD11c、CD103阳性,而BRAF V600E基因突变检测、抗酒石酸酸性磷酸酶染色(TRAP)及骨髓病理Annexin A1免疫组化染色阴性。结论:该类淋巴增殖性疾病是包含多种不同分子生物学特性的淋巴瘤总称,其诊断及鉴别诊断需综合考虑患者的临床特点、相关实验室检查、细胞形态学、流式细胞学检测结果,并合理运用荧光原位杂交(FISH)、分子学及特殊化学及骨髓免疫组化染色等检测方法,少数情况下确诊还需依靠非骨髓累及组织活检。

Objective To investigate the diagnosis and differential diagnosis methods of chronic mature small B-cell lymphoma involving the bone marrow and peripheral blood.Methods The clinical data of 27 patients with mature small B-cell lymphoma involving the bone marrow and peripheral blood(seven subtypes phase IV)who received treatment in the Kunshan Third People's Hospital from February 2015 to June 2021 were retrospectively analyzed.The application value of different detection methods in the diagnosis of mature small B-cell lymphoma involving the bone marrow and peripheral blood was analyzed.Results The majority of patients'peripheral blood was mainly characterized by an increase in the ratio or absolute value of lymphocytes.In terms of cell morphology,mature lymphocytes were mainly small to medium in size.A few bone marrow smears or peripheral blood smears show characteristic changes in cell morphology.Flow cytometry results showed that among the cohort of 15 patients presenting CD5 expression,11 patients had chronic lymphocytic leukemia,1 patient had mantle cell lymphoma,1 patient had splenic diffuse red pulp small B-cell lymphoma,and 2 patients had B-cell chronic lymphoproliferative disorders(unclassified).Among 12 patients presenting no CD5 expression,8 had Waldenström's macroglobulinemia,3 had splenic marginal zone lymphoma,and 1 had follicular lymphoma.Among the 2 patients presenting CD5-CD10 expression,1 patient had follicular lymphoma,and 1 patient had Waldenström's macroglobulinemia.One patient with splenic diffuse red pulp small B-cell lymphoma expressed CD5,CD11c,and CD103 in addition to pan-B-cell markers,while BRAF V600E mutation detection and immunohistochemical staining for tartrate-resistant acid phosphatase and annexin-1A showed negative expression.Conclusion This type of lymphoproliferative disease is a general term for lymphoma that has various different molecular and biological characteristics.Its diagnosis and differential diagnosis need to comprehensively consider the clinical characteristics of the patient,relevant laboratory tests,cell morphology,flow cytometry detection results,reasonable use of fluorescence in situ hybridization,molecular biology,special chemistry,and bone marrow immunohistochemistry.In a few cases,diagnosis of the lymphoproliferative disease still relies on non-bone marrow involvement and tissue biopsy.