Characteristics and Treatment of Hairy Cell Leukaemia

R. Greksák 1
  • 1 Natonal Cancer Insttute, Department of Clinical Oncology, Bratislava, Slovakia


Hairy cell leukaemia (HCL) is a rare chronic indolent lymphoproliferative disease of B-lymphocytes. It always infiltrates the spleen, bone marrow or other organs and is also present in the peripheral blood in more than 95% of cases. The disease takes its name from its characteristic wrinkled surface with hair-like projections. The prognosis of this infaust diagnosis has dramatically changed since the 1980s through more precise diagnosis and efective treatment with purine analogues (1984, 2-deoxycoformy-cine (2-dCF); 1990, 2-chlorodeoxyadenosine (2-CdA)). In our group of 38 patients, we confrmed the diagnostic possibilities and their accuracy; in addition to morphological examination, these include fow cytometric detection of typical surface CD antigens and immunohistochemical recognition of hairy cells in the trephine biopsy. The treatment percentage in the number of response rates (RRs) (100%), complete remissions (CRs) (79%) and overall survival of patients is comparable with previously known data. Overall survival rate without symptoms of disease for more than 10 years after treatment is 87% (33/38). The presence and quantity of minimal residual disease (MRD) after frst-line treatment in bone marrow determine subsequent progression or relapse of the disease, sometimes even occurring many years after the treatment. Probably some new pharmacotherapeutical possibilities (monoclonal antibodies, immunotoxins, tyrosine kinase inhibitors or inhibitors of B-Raf enzyme) could demonstrate the ability to eliminate MRD and cure it in relapsed patients or patients refractory to purine analogues. HCL has become an oncological disease with a relatively good prognosis and long-term survival after standard treatment with purine analogues despite the persistence of indolent MRD in the bone marrow at varying degree of residual infiltration intensity.

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  • [1] Oleske, D. et al. A case-control inquiry into the etiology of hairy cell leukemia. Am J Epidemiol 1985; 121(5): 675-683.

  • [2] Arons, E. et al. VH4-34+ hairy cell leukemia, a new variant with poor prognosis despite standard therapy. Blood 2009; 114(21): 4687-4695.

  • [3] Matutes, E. et al. The immunophenotype of hairy cell leukemia (HCL). Proposal for a scoring system to distinguish HCL from B-cell disorders with hairy or villous lymphocytes. Leukemia Lymph, 1994; 14(1): 57-61.

  • [4] Falini, B. et al.: Simple diagnostic assay for hairy cell leukaemia by immunocytochemical detection of annexin A1 (ANXA1). Lancet 2004; 363(9424): 869-1871.

  • [5] Tiacci, E. et al.: BRAF mutations in hairy-cell leukemia. N Engl J Med 2011; 364(24): 2305-2315.

  • [6] Steis, R.G. et al. Serum solubile IL-2 receptor as a tumor marker in patients with hairy cell leukemia. Blood 1988; 71(5): 1304-1309.

  • [7] Polliack, A. Hairy cell leukemia: biology, clinical diagnosis, unusual manifestations and associated disorders. Rev Clin Exp Hematol 2002; 6(4): 366-388.

  • [8] Hisada, M., et al. Second cancer incidence and cause-specifc mortality among 3104 patients with hairy cell leukaemia: a population-based study. J Natl Cancer Inst 2007; 99(3): 215-222.

  • [9] Seshadri, P. and Seshadri, R. Hairy cell leukemia: treatment prospects. Expert Rev Anticancer Ther 2001; 1(1): 91-98.

  • [10] Golde, D.W. Therapy of hairy-cell leukemia. N Engl J Med 1982; 307(8): 495-496.

  • [11] Frassoldati, A. et al.: Hairy cell leukemia: a clinical review based on 725 cases of the Italian Cooperative Group (IC-GHCL). Leukemia Lymph, 1994; 13(3-4): 307-316.

  • [12] Robak, T. et al. 2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia and hairy cell leukemia variant: 7-year experience in Poland. Eur J Haematol 1999; 62(1): 49-56.

  • [13] Greksák, R. et al. Treatment and prognosis of the patients with hairy cell leukemia, treated in the years 1996 – 2010 at the National Cancer Institute in Bratislava Onkológia 2011; 6(4): 241-245 in slovak.

  • [14] Grever, M.R.: How I treat hairy cell leukemia. Blood 2010; 115(1): 21-28.

  • [15] Greksák, R.: Establishment of the diagnosis and minimal residual disease in hairy cell leukaemia by fow cytom-etry of bone marrow aspirate and imunohistochemical examination of trepanobiopsy. In Onkológia, 2012; 7(6): 406-409 in slovak.

  • [16] Greksák, R. Hairy cell leukaemia – diagnostics, treatment and monoclonal antibodies, In Transfuze Hematol dnes, 2010; 16(3): 158-165 in slovak.


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