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.

Abstract

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 alpha-IFN as first-line treatment; 2) to assess the usefulness of splenectomy as consolidation treatment in patients achieving a satisfactory partial remission (PR) with alpha-IFN, and 3) to explore whether splenectomy in patients achieving complete remission (CR) with alpha-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 alpha-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 alpha-IFNs. At the time of the present analysis, 166 patients (93.8%) were fully evaluable.

RESULTS:

Treatment of HCL patients with alpha-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 alpha-IFNs achieved similar results: the overall response rate (CR + PR + MR) was 92.7%, 97.2%, and 95.3% for patients treated with r-alpha-2a, r-alpha 2b, and alpha-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 alpha-IFN, regardless of the type of alpha-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 alpha-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 alpha-IFN could prolong the progression free survival, its use should be restricted to selected cases.

• Authors: M Federico, A Frassoldati, T Lamparelli, R Foà, M Brugiatelli, L Annino, L Baldini, G Capnist, T Chisesi, P F di Celle
• Journal: Annals of oncology : official journal of the European Society for Medical Oncology (Ann Oncol) Vol. 5 Issue 8 Pg. 725-31 (Oct 1994) ISSN: 0923-7534 [Print] England
• PMID: 7826905 (Publication Type: Clinical Trial, Journal Article, Multicenter Study, Randomized Controlled Trial)

Chemical References

• Interferon alpha-2
• Interferon-alpha
• Recombinant Proteins

Topics

• Adult
• Aged
• Combined Modality Therapy
• Disease-Free Survival
• Female
• Follow-Up Studies
• Humans
• Interferon alpha-2
• Interferon-alpha (administration & dosage, therapeutic use)
• Italy
• Leukemia, Hairy Cell (mortality, surgery, therapy)
• Life Tables
• Male
• Middle Aged
• Prospective Studies
• Recombinant Proteins
• Remission Induction
• Splenectomy
• Survival Rate