T‐cell large granular lymphocyte leukemia

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Abstract
BACKGROUND.: To the authors' knowledge, there is no standard treatment for patients with T‐cell large granular lymphocyte (LGL) leukemia. Available data are limited by patient numbers and coexisting pathologies.METHODS.: The authors report on the use of immunosuppressants (cyclosporin A [CSA] and low‐dose oral methotrexate [MTX] given continuously) and cytotoxic agents in the treatment of 29 patients with T‐cell LGL leukemia age over the past 20 years.RESULTS.: The overall response rate (ORR) to MTX (n = 8 patients) was 85.7% (complete hematologic response [CHR] rate, 14.3%; partial response [PR] rate, 71.4%) with dose‐dependent responses observed and safe usage of doses >10 mg/m2 per week in 2 patients. The ORR to CSA (n = 23 patients) was 78.2% (CHR rate, 30.4%; PR rate, 47.8%). The median time to response for both agents was 1 month. Toxicity, although it was minor in most patients and was more common in the CSA group, included second malignancies in 5 patients. An ORR of 67% (all CHR) was attained with pentostatin (n = 4 patients); recurrences developed after a median of 4.6 years. Successful retreatment with pentostatin was possible but with increasing drug resistance. Cyclophosphamide induced CHR that lasted >7 years with bone marrow clearance in 1 of 4 patients. Alemtuzumab induced a PR in 1 patient who had refractory disease.CONCLUSIONS.: Both MTX and CSA were efficacious in the treatment of T‐cell LGL leukemia but generally required long‐term maintenance therapy. The authors highlight the risks of second malignancies and persistence of bone marrow disease. Although MTX and CSA were effective as first‐line therapy, alemtuzumab and pentostatin merit further investigation, particularly for refractory disease. Cancer 2006 © 2006 American Cancer Society.