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Results in hairy-cell leukemia patients treated with alpha-interferon: predictive prognostic factors
P L Zinzani1, F Lauria, D Raspadori
1Institute of Hematology L. e A. Seràgnoli, University of Bologna, Italy.
Insights
Human lymphoblastoid alpha-interferon (alpha-IFN) shows efficacy in treating hairy cell leukemia (HCL). Continuous alpha-IFN therapy is beneficial for reducing disease progression, especially in patients with high hairy-cell index at diagnosis.
Area of Science:
- Hematology
- Oncology
- Immunotherapy
Background:
- Hairy cell leukemia (HCL) is a rare B-cell malignancy.
- Alpha-interferon (alpha-IFN) has been used in HCL treatment.
- Predictors of treatment response and disease progression in HCL require further investigation.
Purpose of the Study:
- To evaluate the efficacy of human lymphoblastoid alpha-interferon (alpha-IFN) in patients with hairy cell leukemia (HCL).
- To assess the impact of hairy-cell index and maintenance therapy on disease progression.
- To determine the effectiveness of restarting alpha-IFN treatment in progressed patients.
Main Methods:
- Forty-four HCL patients received daily alpha-IFN (3x10^6 Units) for 12-18 months.
- Eighteen patients continued with maintenance therapy (three times/week).
- Disease progression was monitored, and hairy-cell index at diagnosis was analyzed.
Main Results:
- Complete response (18%), partial response (64%), and minor response (18%) were observed.
- Disease progression occurred in 45% of patients, more frequently with high hairy-cell index (>0.50) and without maintenance therapy.
- Restarting alpha-IFN was effective in 9 out of 16 progressed patients.
Conclusions:
- A low hairy-cell index at diagnosis correlates with favorable hematological response.
- Continuous alpha-IFN therapy is effective in reducing HCL progression, particularly for patients with high initial hairy-cell index.
- Maintenance therapy plays a crucial role in preventing disease relapse.
Abstract:
Fourty-four evaluable patients with hairy cell leukemia (HCL) were treated with human lymphoblastoid alpha-interferon (alpha-IFN), at a dose of 3 x 10(6) Units a day for 12-18 months while 18 of them continued to receive a three times per week schedule at the same dose as maintenance treatment. Eighteen percent of patients achieved complete response, 64% partial response, and 18% minor response with a median duration of 37.5, 22.9 and 3.5 months respectively. Twenty patients (45%), all partial or minor responders, subsequently had progression of the disease. The progression occurred more frequently in patients who presented at diagnosis with a hairy-cell index value > 0.50 than in those who presented with a hairy-cell index < 0.50: 14/26 (54%) versus 2/11 (18%) respectively. In addition, the progression rate was more evident in "non-maintained" than in "maintained" patients: 16/26 (61.5%) versus 4/18 (22%). Restarting alpha-IFN treatment in 16 of the 20 progressed patients proved effective only in 9 of them. From these findings it appears that a low hairy-cell index at diagnosis correlates favorably with a good hematological response. Furthermore, continuous therapy with alpha-IFN seems very useful in reducing the progression of the disease, in particular in patients with a very high hairy-cell index at diagnosis.