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Clinical outcomes in T-cell large granular lymphocytic leukaemia: prognostic factors and treatment response.
Br J Haematol. 2020 Jun 09 [Online ahead of print]BJ

Abstract

T-cell large granular lymphocytic leukaemia (T-LGLL) is an incurable leukaemia characterised by clonal proliferation of abnormal cytotoxic T cells that can result in severe neutropenia, transfusion-dependent anaemia and pancytopenia requiring treatment. The most commonly used agents, methotrexate (MTX), cyclophosphamide (Cy) and cyclosporine primarily produce partial remissions (PRs), with few complete responses (CRs). We evaluated the clinical course and treatment response of 60 consecutive patients with T-LGLL to evaluate clinical outcomes and future potential treatment directions. Impaired overall survival was noted among male patients, patients with elevated lactate dehydrogenase, and those without rheumatoid arthritis. Cy was the most efficacious second-line agent, with a 70% overall response rate (ORR; three CR, four PR). All patients who failed frontline MTX responded to second-line Cy. In the relapsed or Cy-refractory setting, alemtuzumab (n = 4) and pentostatin (n = 3) had an ORR of 50% and 66%, respectively, while duvelisib induced a long-term response in one patient. In this large, retrospective analysis, our results suggest Cy is a highly effective therapy for second-line treatment in T-LGLL and should be considered a strong candidate for up-front therapy in select high-risk patients. Prospective studies evaluating pentostatin, alemtuzumab and novel agents, such as duvelisib, are needed for patients with relapsed/refractory T-LGLL.

Authors+Show Affiliations

Department of Internal Medicine, The Ohio State University, Columbus, OH, USA.Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Department of Medical Oncology and Department of Cancer Biology, Sydney Kimmel Cancer Center, Thomas Jefferson University, Philadelphia, PA, USA.Division of Nursing, James Comprehensive Cancer Center, The Ohio State University, Columbus, OH, USA.Department of Pathology, James Comprehensive Cancer Center, The Ohio State University, Columbus, OH, USA.Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Department of Medical Oncology, Sydney Kimmel Cancer Center, Thomas Jefferson University, Philadelphia, PA, USA.Division of Hematology, Department of Internal Medicine, James Comprehensive Cancer Center, The Ohio State University, Columbus, OH, USA.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32519348

Citation

Braunstein, Zachary, et al. "Clinical Outcomes in T-cell Large Granular Lymphocytic Leukaemia: Prognostic Factors and Treatment Response." British Journal of Haematology, 2020.
Braunstein Z, Mishra A, Staub A, et al. Clinical outcomes in T-cell large granular lymphocytic leukaemia: prognostic factors and treatment response. Br J Haematol. 2020.
Braunstein, Z., Mishra, A., Staub, A., Freud, A. G., Porcu, P., & Brammer, J. E. (2020). Clinical outcomes in T-cell large granular lymphocytic leukaemia: prognostic factors and treatment response. British Journal of Haematology. https://doi.org/10.1111/bjh.16808
Braunstein Z, et al. Clinical Outcomes in T-cell Large Granular Lymphocytic Leukaemia: Prognostic Factors and Treatment Response. Br J Haematol. 2020 Jun 9; PubMed PMID: 32519348.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical outcomes in T-cell large granular lymphocytic leukaemia: prognostic factors and treatment response. AU - Braunstein,Zachary, AU - Mishra,Anjali, AU - Staub,Annette, AU - Freud,Aharon G, AU - Porcu,Pierluigi, AU - Brammer,Jonathan E, Y1 - 2020/06/09/ PY - 2020/02/18/received PY - 2020/05/09/accepted PY - 2020/6/11/entrez KW - T cell KW - T-cell large granular lymphocytic leukaemia KW - leukaemia KW - overall response rate KW - therapeutics JF - British journal of haematology JO - Br. J. Haematol. N2 - T-cell large granular lymphocytic leukaemia (T-LGLL) is an incurable leukaemia characterised by clonal proliferation of abnormal cytotoxic T cells that can result in severe neutropenia, transfusion-dependent anaemia and pancytopenia requiring treatment. The most commonly used agents, methotrexate (MTX), cyclophosphamide (Cy) and cyclosporine primarily produce partial remissions (PRs), with few complete responses (CRs). We evaluated the clinical course and treatment response of 60 consecutive patients with T-LGLL to evaluate clinical outcomes and future potential treatment directions. Impaired overall survival was noted among male patients, patients with elevated lactate dehydrogenase, and those without rheumatoid arthritis. Cy was the most efficacious second-line agent, with a 70% overall response rate (ORR; three CR, four PR). All patients who failed frontline MTX responded to second-line Cy. In the relapsed or Cy-refractory setting, alemtuzumab (n = 4) and pentostatin (n = 3) had an ORR of 50% and 66%, respectively, while duvelisib induced a long-term response in one patient. In this large, retrospective analysis, our results suggest Cy is a highly effective therapy for second-line treatment in T-LGLL and should be considered a strong candidate for up-front therapy in select high-risk patients. Prospective studies evaluating pentostatin, alemtuzumab and novel agents, such as duvelisib, are needed for patients with relapsed/refractory T-LGLL. SN - 1365-2141 UR - https://www.unboundmedicine.com/medline/citation/32519348/Clinical_outcomes_in_T-cell_large_granular_lymphocytic_leukaemia:_prognostic_factors_and_treatment_response L2 - https://doi.org/10.1111/bjh.16808 DB - PRIME DP - Unbound Medicine ER -
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