Treatment of T-cell Prolymphocytic Leukemia with Active Disease Criteria
In T-cell prolymphocytic leukemia (T-PLL), treatment is indicated once the disease becomes active. A single qualifying criterion is sufficient to define active disease and to justify initiating therapy.
Active Disease Criteria
Treatment is warranted when at least one of the following is present:
Significant fatigue (ECOG ≥2)
Weight loss >10% in ≤6 months
Drenching night sweats
Fever >38°C (without infection)
Hemoglobin <10 g/dL
Platelets <100 × 10⁹/L
Rapidly enlarging lymph nodes / spleen / liver
Increasing lymphocytosis
Extranodal involvement
Treatment Approach
The approach for active T-PLL involves an antibody-based induction phase, with consolidation considered for patients who achieve a complete remission.
Response Goals
- Absence of lymphadenopathy, splenomegaly, and hepatomegaly
- Circulating lymphocyte count <4 × 10⁹/L
- T-PLL cells <5% of mononuclear cells in bone marrow
References
DOI: 10.1182/blood.2019000402
- Treatment is indicated for patients with active T-PLL and any of the listed disease-related symptoms (Table 4).
- Staging: at least 1 criterion defines active T-PLL (indication for treatment).
- The best first-line treatment to achieve a CR is IV alemtuzumab (anti-CD52) with a ORR higher than 90% and PFS between 8 and 11 months.
- For this reason, patients achieving a complete remission should be considered for consolidation therapy with an allogeneic stem cell transplant.
- Autologous stem cell transplant is an option, particularly for patients without a donor, and has been shown to prolong PFS, but with no long-term survivors.
- Recommendation: IV alemtuzumab induction therapy for 10 to 12 weeks (to achieve best response), followed by consolidation with HSCT where feasible.
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