Long-term results of alpha interferon as initial therapy and splenectomy as consolidation therapy in patients with hairy cell leukemia. Final report from the Italian Cooperative Group for HCL

M. Federico, A. Frassoldati, T. Lamparelli, R. Foa, M. Brugiatelli, L. Annino, L. Baldini, G. Capnist, T. Chisesi, P. F. Di Celle, R. Invernizzi, F. Lauria, M. Truini, L. Resegotti, V. Silingardi, E. E. Damasio

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Purpose: In 1987 the Italian Cooperative Group for the Study of hairy cell leukemia (HCL) started a prospective trial with the following three major aims: 1) to confirm the effectiveness of α-IFN as first-line treatment; 2) to assess the usefulness of splenectomy as consolidation treatment in patients achieving a satisfactory partial remission (PR) with α-IFN, and 3) to explore whether splenectomy in patients achieving complete remission (CR) with α-IFN can reduce the risk of relapse after discontinuation of the drug. Patients and Methods: One-hundred seventy-seven patients with histologically-confirmed HCL were registered in the HCL88-A trial between December 1987 and January 1992. Inclusion criteria included no previous treatment and age less than 66 years. All patients received total doses of 3 MU of α-IFN daily for 12 months except for those who achieved early CR and would stop treatment after 6 or 9 months. Patients could be treated with different α-IFNs. At the time of the present analysis, 166 patients (93.8%) were fully evaluable. Results: Treatment of HCL patients with α-IFN at the onset of the disease resulted in 28 CR (16.9%), 103 PR (62.0%), and 27 Minor Remissions (MR) (16.3%). Patients treated with different α-IFNs achieved similar results: the overall response rate (CR + PR + MR) was 92.7%, 97.2%, and 95.3% for patients treated with r-α-2a, r-α2b, and α-N1, respectively. The presence of a leukemic phase and a poor performance status were associated with a statistically significant lower response rate. Patients who were randomly assigned and underwent splenectomy after achieving a PR had a better but not significant 4-year progression-free survival than cases randomized for observation (53% vs. 22%, p = 0.116). Overall, 5 patients died after study entry, with an actuarial 5-year survival rate of 96% for the entire group of 166 patients: After a mean follow-up time of 38 months, only one second malignancy has been recorded. Conclusions: Initial therapy with α-IFN, regardless of the type of α-IFN used, induces satisfactory responses in the majority of patients with HCL, but in most instances discontinuation of treatment results in recurrence of disease. In most cases α-IFN improves the performance status of patients and favors a satisfactory bone marrow recovery and thus could still play a role in the initial management of the disease. Although splenectomy following α-IFN could prolong the progression free survival, its use should be restricted to selected cases.

Original languageEnglish
Pages (from-to)725-731
Number of pages7
JournalAnnals of Oncology
Issue number8
Publication statusPublished - 1994


  • α-Interferon
  • 2-Chlorodeoxyadenosine
  • Hairy cell leukemia
  • Splenectomy

ASJC Scopus subject areas

  • Cancer Research
  • Statistics, Probability and Uncertainty
  • Applied Mathematics
  • Hematology
  • Oncology
  • Public Health, Environmental and Occupational Health
  • Neuropsychology and Physiological Psychology


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