46例脾脏淋巴组织肿瘤病理形态免疫表型及分子遗传学探讨

The Studies on Pathomorphology,Immunophenotype and Molecule Genetics in 46 Cases of Lymphoid Tissue Tumours of Spleen

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 features of pathomorphology, immunophenotype and molecular genetics and the differential diagnosis of lymphoid tissue tumours of spleen(LTTS). Methods: Paraffin-embedded-section and HE staining were made for morphologic analysis in 46 cases of LTTS, immunophenotype was detected with S-P immunohistochemical(IHC) method and 21 M-Abs in 43 cases, and polymerase chain reaction(PCR)was used to detect IgH and TCR gene rearrangement in 16 cases of primary splenic lymphoma. Results: Of the 43 LTTS, 8 cases (18.6%) were of T cell origin(PTL-N 4, HS-TL 4), 35 (81.4%) were B cell (HCL 13, SMZL 5, SLVL 2, FL 4, MCL 2 SLL 2, DLBL 4, TR-LBL 3), and 3 ALLs (autopsy) were not immunotyped. IgH gene rearrangement was found in 8 of 10 cases and TCR gene rearrangement was found in 5 of 6 cases. Mean weight of spleens was 2227.08±324.69g (range from 196g to 6000g). 6 cases were found splenic obstruction. Conclusions: Each type of LTTS may have its unique macrography and histopathology characteristics. It is important to differentiate HCL or ALLs from PLS by PB and BM detections, and to make diagnosis and differential diagnosis of LTTS by PCR and IHC.