Hairy B-cell lymphoproliferative disorder and its differential diagnosis: a case with long-term follow-up

Main Article Content

Kensuke Matsuda
Yosuke Matsumoto
Mihiko Yoshida
Kazuho Shimura
Hiroto Kaneko
Tohru Inaba
Shigeo Horiike
Junya Kuroda
Masafumi Taniwaki



Hairy B-cell lymphoproliferative disorder (HBLD) is one of chronic polyclonal B-cell lymphocytosis. We report a 47-year-old female Japanese patient diagnosed as having HBLD based on lymphocytosis with hairy cell appearance and characteristic phenotypes including CD11c+, and without B-cell monoclonalities. She was a non-smoker, and possessed HLA-DR4. She has been closely followed up without treatment and lymphoma development for over five years. Although this disease is quite rare and has been reported, to our knowledge, in only 13 Japanese cases, an accurate diagnosis, particularly differential diagnosis from persistent polyclonal B-cell lymphocytosis or hairy cell leukemia-Japanese variant is essential for the prevention of unnecessary treatments.


Download data is not yet available.
Abstract 1742 | PDF Downloads 491 HTML Downloads 805


1. Beutler E, Lichtman MA, Coller BS, Kipps TJ, Seligsohn U, editors. Williams Hematology, sixth ed. New York: McGraw Hill; 2001:pp969-76.

2. Gordon DS, Jones BM, Browning SW, Spira TJ, Lawrence DN. Persistent polyclonal lymphocytosis of B lymphocytes. N Engl J Med 1982;307:232-6.

3. Troussard X, Valensi F, Debert C, Maynadie M, Schillinger F, Bonnet P, Macintyre EA, Flandrin G. Persistent polyclonal lymphocytosis with binucleated B lymphocytes: a genetic predisposition. Br J Haematol 1994;88:275-80.

4. Mossafa H, Malaure H, Maynadie M, Valensi F, Schillinger F, Garand R, Jung G, Flandrin G, Troussard X. Persistent polyclonal B lymphocytosis with binucleated lymphocytes: a study of 25 cases. Groupe Français d'Hématologie Cellulaire. Br J Haematol 1999;104:486-93.

5. Schmidt-Hieber M, Burmeister T, Weimann A, Nagorsen D, Hofmann WK, Thiel E, Schwartz S. Combined automated cell and flow cytometric analysis enables recognition of persistent polyclonal B-cell lymphocytosis (PPBL), a study of 25 patients. Ann Hematol 2008;87:829-36.

6. Cornet E, Lesesve JF, Mossafa H, Sébahoun G, Levy V, Davi F, Troussard X; Groupe Français d'Hématologie Cellulaire (GFHC). Long-term follow-up of 111 patients with persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes. Leukemia 2009;23:419-22.

7. Matsue K, Nishi H, Onozawa S, Itoh M, Tsukuda K, Yamaguchi M, Nakao S, Kashimura M. Polyclonal B cell chronic lymphoproliferative disease with hairy cell morphology: a case report and clonal studies. Am J Hematol 1996;51:141-6.

8. Machii T, Yamaguchi M, Inoue R, Tokumine Y, Kuratsune H, Nagai H, Fukuda S, Furuyama K, Yamada O, Yahata Y, Kitani T. Polyclonal B-cell lymphocytosis with features resembling hairy cell leukemia-Japanese variant. Blood 1997;89:2008-14.

9. Troussard X, Mossafa H, Flandrin G. Identity between hairy B-cell lymphoproliferative disorder and persistent polyclonal B lymphocytosis? Blood 1997;90:2110-3.

10. Kanbayashi H, Nagata K, Tanaka T, Matsuda S, Sakuma H, Maruyama Y, Machii T. Polyclonal B-cell lymphocytosis with clinical and hematological features resembling hairy cell leukemia. Jpn J Clin Hematol 1998;39:493-8 in Japanese.

11. Yagi Y, Sakabe H, Kaninoki R, Yoshikawa K, Inoue T, Fujiyama Y, Machii T. A hairy B-cell lymphoproliferative disorder resembling hairy cell leukemia. Jpn J Clin Hematol 2004;45:312-5 in Japanese.

12. Nakahashi H, Hashimoto Y, Yamane A, Irisawa H, Yokohama A, Saitoh T, Hanada H, Matsushima T, Tsukamoto N, Karasawa M, Murakamai H, Nojima Y. Polyclonal B-cell lymphocytosis with hairy cell appearance: hairy B-cell lymphoproliferative disorder. Jpn J Clin Hematol 2007;48:647-51 in Japanese.

13. Takeshita T, Owatari S, Otsuka M, Hanada S. Remarkable response to cladribine in the treatment for hairy B cell lymphoproliferative disorder. J Jpn Soc Int Med 2007;96:1712-14 in Japanese.

14. Okamoto A, Inaba T, Fujita N. The role of interleukin-6 in a patient with polyclonal hairy B-cell lymphoproliferative disorder: a case report. Laboratory Hematology 2007;13:124-7.

15. van Dongen JJ, Langerak AW, Brüggemann M, Evans PA, Hummel M, Lavender FL, Delabesse E, Davi F, Schuuring E, García-Sanz R, van Krieken JH, Droese J, González D, Bastard C, White HE, Spaargaren M, González M, Parreira A, Smith JL, Morgan GJ, Kneba M, Macintyre EA. Design and standardization of PCR primers and protocols for detection of clonal immunoglobulin and T-cell receptor gene recombinations in suspect lymphoproliferations: report of the BIOMED-2 Concerted Action BMH4-CT98-3936. Leukemia 2003;17:2257-317.

16. Tiacci E, Trifonov V, Schiavoni G, Holmes A, Kern W, Martelli MP, Pucciarini A, Bigerna B, Pacini R, Wells VA, Sportoletti P, Pettirossi V, Mannucci R, Elliott O, Liso A, Ambrosetti A, Pulsoni A, Forconi F, Trentin L, Semenzato G, Inghirami G, Capponi M, Di Raimondo F, Patti C, Arcaini L, Musto P, Pileri S, Haferlach C, Schnittger S, Pizzolo G, Foà R, Farinelli L, Haferlach T, Pasqualucci L, Rabadan R, Falini B. BRAF mutations in hairy-cell leukemia. N Engl J Med 2011;364:2305-15.

17. Katayama I, Mochino T, Honma T, Fukuda M. Hairy cell leukemia: a comparative study of Japanese and non-Japanese patients. Semin Oncol 1984;11(4 Suppl. 2) 486-92.

18. Machii T, Tokumine Y, Inoue R, Kitani T, Predominance of a distinct subtype of hairy cell leukemia in Japan. Leukemia 1993;7:181-6.

19. Delage R, Roy J, Jacques L, Bernier V, Delâge JM, Darveau A. Multiple bcl-2/Ig gene rearrangements in persistent polyclonal B-cell lymphocytosis. Br J Haematol 1997;97:589-95.

20. Chan MA, Benedict SH, Carstairs KC, Francombe WH, Gelfand EW. Expansion of B lymphocytes with an unusual immunoglobulin rearrangement associated with atypical lymphocytosis and cigarette smoking. Am J Respair Cell Mol Biol 1990;2:549-52.