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目的:探讨LTTS的病理形态,免疫表型及分子遗传学特点及其鉴别诊断。方法:46例脾标本作常规石蜡切片、HE及网状纤维染色,43例用21种单抗SP法免疫组化染色,16例用PCR检测IgH和TCR基因重排。结果:43例LTTS中T细胞性8例(18.6%),其中PTL-NS4例,HS-TL4例;B细胞性35例(81.4%),其中HCL13例,SMZL5例,SLVL2例,FL4例,MCL2例,SLL2例,DLBL4例,TR-LBL3例;3例ALL未做免疫分型。10例B细胞淋巴瘤IgH基因重排检出8例,6例T细胞的TCR基因重排检出5例。脾平均重2227.08±324.69g(范围196～6000g),脾梗死6例。结论:LTTS具有明显的大体与镜下病理形态特征,结合血象和骨髓象有利于将HCL和ALL与脾淋巴瘤相鉴别,结合免疫组化和PCR检测有利于诊断与鉴别诊断。
Objective: To investigate the pathological features, immunophenotype and molecular genetics of LTTS and its differential diagnosis. Methods: Forty-six spleen specimens were stained with HE and reticular fibers, 43 of them were stained with 21 monoclonal antibodies (SPs) and 16 of them were examined by PCR for IgH and TCR rearrangements. RESULTS: Among the 43 cases of LTTS, 8 cases were T cells (18.6%), including 4 cases of PTL-NS, 4 cases of HS-TL and 35 cases of B cell (81.4%), including 13 cases of HCL, 5 cases of SMZL, 2 cases of SLVL, 4 cases of FL, MCL2 cases, SLL2 cases, DLBL4 cases, TR-LBL3 cases; 3 cases of ALL did not do immunophenotyping. In 10 cases of B-cell lymphoma, IgH gene rearrangement was detected in 8 cases and 6 cases of T cells were detected by TCR gene rearrangement in 5 cases. Spleen average weight 2227.08 ± 324.69g (range 196 ~ 6000g), spleen infarction in 6 cases. CONCLUSIONS: LTTS has obvious gross and microscopic pathological features. Combining blood and bone marrow imaging is helpful for differentiating HCL and ALL from splenic lymphoma. Combined with immunohistochemistry and PCR, LTTS is helpful for the diagnosis and differential diagnosis.