A rare case of B-lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） translocation  

作　　者：Xiaofeng Shi Rong Ba Haiyan You Qian Jiang Jiansong Huang Jianhua Mao Lanxiu Han Shuo Zhang Qin Zhuang Xianqiu Yu Lixia Wang Yun Wang Dongya Li Wei Zhu Yong Zhang Yan Zhu Xiaodong Xi 

机构地区：[1]Department of Hematology, Affiliated Hospital of Jiangsu University, Zhenjiang 212001, China [2]The Center of Thrombosis, Affiliated Hospital of Jiangsu University, Zhenjiang 212001, China [3]State Key Laboratory of Medical Genomics, Shanghai Institute of Hematology, Collaborative Innovation Center of Hematology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China [4]Institute of Hematology, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310003, China [5]School of Medicine, Jiangsu University, Zhenjiang 212013, China [6]Blood Cell Development and Function Program Fox Chase Cancer Center, 333 Cottman Avenue, Room 364, Philadelphia, PA 19111-2497, USA

出　　处：《Frontiers of Medicine》2018年第3期324-329,共6页医学前沿（英文版）

基　　金：This work was supported in part by grants from National Natural Science Foundation of China （No. 81670127） and Natural Science Foundation of Jiangsu Province （No. BK20150474）, and Xiaofeng Shi is a member of medical key personnel of Zhenjiang Municipality and young medical personnel of Jiangsu Province.

摘　　要：Splenic lymphoma with villous lymphocytes （SLVL） or splenic marginal zone lymphoma with circulating villous lymphocytes is rare, and prolymphocytic transformation of SLVL is rarer. At present, only one case of SLVL with t（8;14）（q24;q32） translocation has been reported. In this study, we report a case of B- lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） chromosome translocation that we inclined to SLVL with a prolymphocytic transformation. A 73-year-old female showed marked hepatosplenomegaly and high lymphocytosis （lymphocytes 〉 200 × 10^9/L）. The abnormal lymphocytes had short coarse villi and round nuclei with prominent nucleoli. The immunophenotypes showed CD19^＋, CD20^＋, HLA- DR^＋, CD22^＋, CD5^＋, Kappa^＋, CD25^dim, CD71^dim, Lambda, CD7, CD10-, CD23 , CD34, CD33, CD13 , CD14, CDll7, CD64, CD103, and CD11c-. The karyotype showed complex abnormality： 46XX,＋ 3,-10, t（8;14）（q24; q32）lll]/46XX[9]. The cytoplasmic projection, immunological characteristics, and trisomy 3 chromosome abnormality supported the diagnosis of SLVL. However, the presence of prominent nucleoli and high lymphocytosis suggested prolymphocytic transformation, probably as a result of t（8,14） chromosome translocation. In this report, we described an unusual case of B-lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） translocation, which could provide help in the diagnosis and differential diagnosis of B-lymphocytic proliferative diseases.Splenic lymphoma with villous lymphocytes （SLVL） or splenic marginal zone lymphoma with circulating villous lymphocytes is rare, and prolymphocytic transformation of SLVL is rarer. At present, only one case of SLVL with t（8;14）（q24;q32） translocation has been reported. In this study, we report a case of B- lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） chromosome translocation that we inclined to SLVL with a prolymphocytic transformation. A 73-year-old female showed marked hepatosplenomegaly and high lymphocytosis （lymphocytes 〉 200 × 10^9/L）. The abnormal lymphocytes had short coarse villi and round nuclei with prominent nucleoli. The immunophenotypes showed CD19^＋, CD20^＋, HLA- DR^＋, CD22^＋, CD5^＋, Kappa^＋, CD25^dim, CD71^dim, Lambda, CD7, CD10-, CD23 , CD34, CD33, CD13 , CD14, CDll7, CD64, CD103, and CD11c-. The karyotype showed complex abnormality： 46XX,＋ 3,-10, t（8;14）（q24; q32）lll]/46XX[9]. The cytoplasmic projection, immunological characteristics, and trisomy 3 chromosome abnormality supported the diagnosis of SLVL. However, the presence of prominent nucleoli and high lymphocytosis suggested prolymphocytic transformation, probably as a result of t（8,14） chromosome translocation. In this report, we described an unusual case of B-lymphoproliferative disorder with villous lymphocytes harboring t（8;14）（q24;q32） translocation, which could provide help in the diagnosis and differential diagnosis of B-lymphocytic proliferative diseases.

关 键 词：splenic lymphoma with villous lymphocytes splenic marginal zone lymphoma transformation chromosometranslocation 

分 类 号：S827.1[农业科学—畜牧学] X591[农业科学—畜牧兽医]