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
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.
Camacho FI, Mollejo M, Mateo MS, Algara P, Navas C, Hernández JM, Santoja C, Solé F, Sánchez-Beato M, Piris MA. Progression to large B-cell lymphoma in splenic marginal zone lymphoma: a description of a series of 12 cases. Am J Surg Pathol 2001; 25(10): 1268–1276 https://doi.org/10.1097/00000478-200110000-00007
pmid: 11688461
2
Dungarwalla M, Appiah-Cubi S, Kulkarni S, Saso R, Wotherspoon A, Osuji N, Swansbury J, Cunningham DC, Catovsky D, Dearden CE, Matutes E. High-grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis. Br J Haematol 2008; 143(1): 71–74 https://doi.org/10.1111/j.1365-2141.2008.07301.x
pmid: 18671706
3
Hoehn D, Miranda RN, Kanagal-Shamanna R, Lin P, Medeiros LJ. Splenic B-cell lymphomas with more than 55% prolymphocytes in blood: evidence for prolymphocytoid transformation. Hum Pathol 2012; 43(11): 1828–1838 https://doi.org/10.1016/j.humpath.2012.01.003
pmid: 22520947
4
Traverse-Glehen A, Baseggio L, Bauchu EC, Morel D, Gazzo S, Ffrench M, Verney A, Rolland D, Thieblemont C, Magaud JP, Salles G, Coiffier B, Berger F, Felman P. Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity? Blood 2008; 111(4): 2253–2260 https://doi.org/10.1182/blood-2007-07-098848
pmid: 18042795
5
Troussard X, Mauvieux L, Radford-Weiss I, Rack K, Valensi F, Garand R, Vekemans M, Flandrin G, Macintyre EA. Genetic analysis of splenic lymphoma with villous lymphocytes: a Groupe Français d’Hématologie Cellulaire (GFHC) study. Br J Haematol 1998; 101(4): 712–721 https://doi.org/10.1046/j.1365-2141.1998.00764.x
pmid: 9674745
6
Sonu R, Gregg J, Chen M. Splenic marginal zone lymphoma with t(8;14)(q24.1;q32)/MYC rearrangement. J Hematop 2012; 5(3): 141–147 https://doi.org/10.1007/s12308-011-0124-6
7
Visser L, Shaw A, Slupsky J, Vos H, Poppema S. Monoclonal antibodies reactive with hairy cell leukemia. Blood 1989; 74(1): 320–325
pmid: 2665837
8
Shao H, Calvo KR, Grönborg M, Tembhare PR, Kreitman RJ, Stetler-Stevenson M, Yuan CM. Distinguishing hairy cell leukemia variant from hairy cell leukemia: development and validation of diagnostic criteria. Leuk Res 2013; 37(4): 401–409 https://doi.org/10.1016/j.leukres.2012.11.021
pmid: 23347903
Cornet E, Delmer A, Feugier P, Garnache-Ottou F, Ghez D, Leblond V, Levy V, Maloisel F, Re D, Zini JM, Troussard X;French Society of Haematology. Recommendations of the SFH (French Society of Haematology) for the diagnosis, treatment and follow-up of hairy cell leukaemia. Ann Hematol 2014; 93(12): 1977–1983 https://doi.org/10.1007/s00277-014-2140-y
pmid: 24994538
11
Troussard X, Valensi F, Duchayne E, Garand R, Felman P, Tulliez M, Henry-Amar M, Bryon PA, Flandrin G. Splenic lymphoma with villous lymphocytes: clinical presentation, biology and prognostic factors in a series of 100 patients. Groupe Francais d’Hématologie Cellulaire (GFHC). Br J Haematol 1996; 93(3): 731–736 https://doi.org/10.1046/j.1365-2141.1996.d01-1711.x
pmid: 8652403
12
Matutes E, Morilla R, Owusu-Ankomah K, Houliham A, Meeus P, Catovsky D. The immunophenotype of hairy cell leukemia (HCL). Proposal for a scoring system to distinguish HCL from B-cell disorders with hairy or villous lymphocytes. Leuk Lymphoma 1994;14 (Suppl 1):57–61
pmid: 7820054
13
Tierens A, Delabie J, Malecka A, Wang J, Gruszka-Westwood A, Catovsky D, Matutes E. Splenic marginal zone lymphoma with villous lymphocytes shows on-going immunoglobulin gene mutations. Am J Pathol 2003; 162(2): 681–689 https://doi.org/10.1016/S0002-9440(10)63862-X
pmid: 12547726
14
Salido M, Baró C, Oscier D, Stamatopoulos K, Dierlamm J, Matutes E, Traverse-Glehen A, Berger F, Felman P, Thieblemont C, Gesk S, Athanasiadou A, Davis Z, Gardiner A, Milla F, Ferrer A, Mollejo M, Calasanz MJ, Florensa L, Espinet B, Luño E, Wlodarska I, Verhoef G, García-Granero M, Salar A, Papadaki T, Serrano S, Piris MA, Solé F. Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group. Blood 2010; 116(9): 1479–1488 https://doi.org/10.1182/blood-2010-02-267476
pmid: 20479288
15
Matutes E, Morilla R, Owusu-Ankomah K, Houlihan A, Catovsky D. The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders. Blood 1994; 83(6): 1558–1562
pmid: 8123845
16
Melo JV, Hegde U, Parreira A, Thompson I, Lampert IA, Catovsky D. Splenic B cell lymphoma with circulating villous lymphocytes: differential diagnosis of B cell leukaemias with large spleens. J Clin Pathol 1987; 40(6): 642–651 https://doi.org/10.1136/jcp.40.6.642
pmid: 3497180
17
Melo JV, Wardle J, Chetty M, England J, Lewis SM, Galton DA, Catovsky D. The relationship between chronic lymphocytic leukaemia and prolymphocytic leukaemia. III. Evaluation of cell size by morphology and volume measurements. Br J Haematol 1986; 64(3): 469–478 https://doi.org/10.1111/j.1365-2141.1986.tb02202.x
pmid: 3466642
Lichtman MA, Kipps TJ, Seligsohn U, Kaushansky K, Prchal JT. Chapter 95: Hairy Cell Leukemia. In: Kaushansky K, Lichtman MA, Kipps TJ. Williams Hematology. Eighth Edition. United States: The McGraw-Hill Companies, Inc. 2010; 1485–1486
20
Heintel D, Streubel B, Welzel N, Le T, Schwarzinger I, Haas OA, Simonitsch I, Lechner K, Jaeger U. Burkitt lymphoma following splenic marginal zone lymphoma: evidence for two independent B-cell clones. Cancer Genet Cytogenet 2003; 141(1): 86–88 https://doi.org/10.1016/S0165-4608(02)00724-0
pmid: 12581905
21
Flatley E, Chen AI, Zhao X, Jaffe ES, Dunlap JB, Pittaluga S, Abdullah S, Olson SB, Spurgeon SE, Fan G. Aberrations of MYC are a common event in B-cell prolymphocytic leukemia. Am J Clin Pathol 2014; 142(3): 347–354 https://doi.org/10.1309/AJCPUBHM8U7ZFLOB
pmid: 25125625
22
Damm F, Mylonas E, Cosson A, Yoshida K, Della Valle V, Mouly E, Diop M, Scourzic L, Shiraishi Y, Chiba K, Tanaka H, Miyano S, Kikushige Y, Davi F, Lambert J, Gautheret D, Merle-Béral H, Sutton L, Dessen P, Solary E, Akashi K, Vainchenker W, Mercher T, Droin N, Ogawa S, Nguyen-Khac F, Bernard OA. Acquired initiating mutations in early hematopoietic cells of CLL patients. Cancer Discov 2014; 4(9): 1088–1101 https://doi.org/10.1158/2159-8290.CD-14-0104
pmid: 24920063
23
Chung SS, Kim E, Park JH, Chung YR, Lito P, Teruya-Feldstein J, Hu W, Beguelin W, Monette S, Duy C, Rampal R, Telis L, Patel M, Kim MK, Huberman K, Bouvier N, Berger MF, Melnick AM, Rosen N, Tallman MS, Park CY, Abdel-Wahab O. Hematopoietic stem cell origin of BRAFV600E mutations in hairy cell leukemia. Sci Transl Med 2014; 6(238): 238ra71 https://doi.org/10.1126/scitranslmed.3008004
pmid: 24871132
24
Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani R, Ghielmini M, Salles GA, Zelenetz AD, Jaffe ES. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127(20): 2375–2390 https://doi.org/10.1182/blood-2016-01-643569
pmid: 26980727