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Frontiers of Medicine

ISSN 2095-0217

ISSN 2095-0225(Online)

CN 11-5983/R

Postal Subscription Code 80-967

2018 Impact Factor: 1.847

Front. Med.    2018, Vol. 12 Issue (3) : 324-329    https://doi.org/10.1007/s11684-017-0558-z
CASE REPORT |
A rare case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) translocation
Xiaofeng Shi1,2,3, Rong Ba1, Haiyan You1, Qian Jiang1, Jiansong Huang4, Jianhua Mao3, Lanxiu Han1, Shuo Zhang1, Qin Zhuang1, Xianqiu Yu1, Lixia Wang1, Yun Wang1, Dongya Li1, Wei Zhu5, Yong Zhang6, Yan Zhu1(), Xiaodong Xi3()
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
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Abstract

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>200×109/L). The abnormal lymphocytes had short coarse villi and round nuclei with prominent nucleoli. The immunophenotypes showed CD19+, CD20+, HLA-DR+, CD22+, CD5+, Kappa+, CD25dim, CD71dim, Lambda, CD7, CD10, CD23, CD34, CD33, CD13, CD14, CD117, CD64, CD103, and CD11c. The karyotype showed complex abnormality: 46XX,+3,−10, t(8;14)(q24;q32)[11]/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.

Keywords splenic lymphoma with villous lymphocytes      splenic marginal zone lymphoma      transformation      chromosome translocation     
Corresponding Authors: Yan Zhu,Xiaodong Xi   
Online First Date: 31 October 2017    Issue Date: 04 May 2018
 Cite this article:   
Xiaofeng Shi,Rong Ba,Haiyan You, et al. A rare case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) translocation[J]. Front. Med., 2018, 12(3): 324-329.
 URL:  
http://academic.hep.com.cn/fmd/EN/10.1007/s11684-017-0558-z
http://academic.hep.com.cn/fmd/EN/Y2018/V12/I3/324
Fig.1  (A) Blood film. Villous lymphocytes (Wright’s stain, magnification 1000×). (B) Magnified image showing the short coarse cytoplasmic projections, round nucleus with less condensed chromatin, and one or two nucleoli. One smudge cell, which was a result of mechanical fragility from the shear forces generated in blood film preparation, was notable. (Wright’s stain, magnification 4000×.) (C) Negative for peroxidase stain in villous lymphocytes (strong positive in a neutrophil). (D) Negative for specific esterase stain in villous lymphocytes (strong positive in a neutrophil).
Fig.2  Immunophenotypes using flow cytometry. 96% leukocytes were abnormal and expressed CD19 (65%), CD20 (89%), HLA-DR (94%), CD22 (94%), CD5high (97%), Kappahigh (99%), CD25dim (41%), and CD71dim (53%), but did not express Lambda, CD7, CD10, CD23, CD34, CD33, CD13, CD14, CD117, CD64, CD103, and CD11c.
Fig.3  (A) Mitotic figure. (B) Karyotype showed complex chromosomal abnormality: 46XX, +3,−10, t(8;14)(q24;q32)[11]/46XX[9]. One chromosome 10 is lost randomly.
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