Hairy cell leukemia
accompanying life-threatening infection, socioeco- nomic factors, and other unusual circumstances.
Splenectomy
Splenectomy was the standard of care before 1984. Splenectomy had no effect on bone marrow infiltra- tion, pattern, or fibrosis. Interpretation of the data is complex because standard response criteria that are now in place were different prior to therapeutic interventions which improved the bone marrow pattern. There were eight major reports on splenect- omy [1]. The complete and partial remission rates were 60–100%. Spleens less than 4 cm below the costal margin had an increase in hemoglobin only [5]. Survival data were complex and difficult to interpret because of the indications for splenectomy, intrinsic patient population characteristics, timing of splenectomy, and patient selection, and the data were predominantly retrospective. Jansen et al. in an early report reported median survival of 30–36 months with no difference in survival for no splenectomy (55%) or splenectomy (75%) at 4 years [6]. In a subsequent report there was a 48-month median survival, with 38% survival at 3 years without splenectomy versus 55% with splenectomy [5]. Flandrin et al. reported a retrospective statistically significant difference in survival at 5 years after splenectomy versus no splenectomy [7]. Damasio et al. reported a median survival of 33 months in patients with splenectomy versus 27 months for patients without splenectomy [8].
Recombinant interferon-a
Quesada and associates initially reported activity with interferon-a in hairy cell leukemia [9]. In a follow-up report, 30 patients had a complete remission and 56% a partial remission, with all patients having improvement and normalization of peripheral blood counts. Golomb and associates reported on 64 patients, 61 of whom had splenectomy, with a complete remission of 5% and partial remission of 70% [10]. Rai and associates reported on 55 patients with a 24% complete remission rate and partial remission rate of 49% [11]. The overall survival at 6 years was 83%. Troussard and Flandrin reported an interferon maintenance program where patients were randomized to observation or maintenance interfer- on-a [12]. Sixty-six percent (37/56) of patients in the observation arm relapsed compared to 0% (0/28) of patients in the maintenance treatment arm at a median follow-up of 30 months. Interferon-a- 2a and -a- 2b were the first biologic
response modifiers approved by the US Food and Drug Administration. There were true bone marrow
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responses. Patients with advanced disease re- sponded. The complete blood count normalized. There were decreased hospital days. There was a significant impact on transfusion requirements. The absolute monocyte count improved significantly in patients who responded. Bone marrow fibrosis was also reversed. In patients who responded, the number of hairy cells decreased in the first week, the platelet count increased in 2 months, and the hemoglobin level increased in 4 months. The responses were predominantly partial remission, with rates of 69–87% reported. Smalley et al. reported a controlled randomized trial with a small number of patients [13]. Patients were randomized to receive recombinant interferon-a versus splenect- omy. The median time-to-treatment failure was 18 months in the interferon group and 1 month in the splenectomy group.
Conclusion
Although newer approaches are now available, alternative therapeutic interventions for some pa- tients with relapsed/refractory disease include ob- servation, splenectomy, and recombinant interferon.
Potential conflict of interest: Disclosure forms provided by the authors are available with the full text of this article at
www.informahealthcare.com/lal.
References
1. Habermann TM. Splenectomy, interferon, and treatments of historical interest in hairy cell leukemia. Hematol Oncol Clin North Am 2006;20:1075–1086.
2. Golomb HM. Progress report on chlorambucil therapy in post-splenectomy patients with progressive hairy cell leukemia. Blood 1981;57:464–467.
3. Albain KS, LeBeau MM, Vardiman JW, et al. Development of dysmyelopoietic syndrome in a hairy cell leukemia patient treated with chlorambucil: cytogenetic and morphologic evaluation. Cancer Genet Cytogenet 1983;8:107–115.
4. Golomb HM, Vardiman JW. Response to splenectomy in 65 patients with hairy cell leukemia: an evaluation of spleen weight and bone marrow involvement. Blood 1983;61:349–352.
5. Jansen J, Hermans J. Splenectomy in hairy cell leukemia: a retrospective multicenter analysis. Cancer 1981;47:2066– 2076.
6. Jansen J, Hermans J, Remme J, et al. Hairy cell leukemia: clinical features and effects of splenectomy. Scand J Haematol 1978;21:60–71.
7. Flandrin G, Sigaux F, Sebhoun G, et al. Hairy cell leukemia: clinical presentation and follow-up of 211 patients. Semin Oncol 1984;11(Suppl. 2):458–471.
8. Damasio E, Spriano M, Repetto M, et al. Hairy cell leukemia: a retrospective study of 235 cases by the Italian Cooperative Group (ICGHCL) according to Jansen’s clinical staging system. Acta Haematol 1984;72:326–334.
9. Quesada JR, Rueben J, Manning JT, et al. Alpha interferon for induction of remission in hairy cell leukemia. N Engl J Med 1984;310:15–18.
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