Ou Jinping, Yang Lu, Ren Limin, Tang Xiuying, Li Ting, Wu Shulan
Department of Hematology, The First Hospital of Peking University, Beijing 100034, China.
Zhonghua Nei Ke Za Zhi. 2002 Jan;41(1):28-30.
To deepen the understanding of splenic marginal zone lymphoma (SMZL) and improve the level of diagnosis and therapy.
A typical case of SMZL, a 61 year old female with lymphocytosis and splenomegaly found fortuitously, was reported. The pathologic, immunologic and genetic features of tumor cells in peripheral blood, bone marrow and spleen were studied with light microscopy, phase contrast microscopy, scanning electron microscopy, immunohistochemical method, flow cytometry, G chromosome banding technique and PCR for studying the pattern of IgH gene rearrangement.
The spleen was large with uniform parenchyma and smooth surface. There were multiple small gray-white nodules on sections. Histologically, the neoplastic cells replaced the marginal and mantle zones with complete replacement of germinal centers in the white pulp. The neoplastic cells were predominantly of small to medium size with oval or slightly irregular nuclei. Lymph nodes in the splenic hilum were infiltrated by tumor cells. Immunophenotypic analysis demonstrated that the lymphocytes in the bone marrow expressed CD(20), HLA-DR, CD(45) RA and bcl-2. The monoclonal pattern of IgH gene rearrangement in peripheral blood and bone marrow was found to be the same as that in spleen. After splenectomy, COP chemotherapy and IFNalpha-2a were given and the abnormally increased lymphocytes decreased to normal level. Seven months later the monoclonal rearranged immunoglobulin heavy chain gene pattern changed to polyclonal pattern.
Splenomegaly, lymphocytosis in peripheral blood and bone marrow without lymph node enlargement and leukocytosis are clinical characters of SMZL. Presence of monoclonal rearranged IgH gene is in favor of the diagnosis. Splenectomy should be done earlier in suspicious patients to avoid malignant transformation.
加深对脾边缘区淋巴瘤（SMZL）的认识，提高诊断和治疗水平。
报告1例典型的SMZL病例，为1名61岁女性，偶然发现淋巴细胞增多和脾肿大。采用光学显微镜、相差显微镜、扫描电子显微镜、免疫组织化学方法、流式细胞术、G染色体显带技术及PCR研究外周血、骨髓和脾脏肿瘤细胞的病理、免疫和遗传学特征，以研究IgH基因重排模式。
脾脏肿大，实质均匀，表面光滑。切片上有多个灰白色小结节。组织学上，肿瘤细胞取代了边缘区和套区，白髓生发中心完全被取代。肿瘤细胞主要为中小细胞，核呈椭圆形或略不规则。脾门淋巴结被肿瘤细胞浸润。免疫表型分析显示骨髓中的淋巴细胞表达CD(20)、HLA-DR、CD(45)RA和bcl-2。外周血和骨髓中IgH基因重排的单克隆模式与脾脏相同。脾切除术后给予COP化疗和干扰素α-2a，异常增多的淋巴细胞降至正常水平。7个月后，单克隆重排免疫球蛋白重链基因模式转变为多克隆模式。
脾肿大、外周血和骨髓淋巴细胞增多而无淋巴结肿大及白细胞增多是SMZL的临床特征。单克隆重排IgH基因的存在有助于诊断。对可疑患者应尽早行脾切除术以避免恶变。
