E Poullot1, R Zambello2, F Leblanc3, B Bareau4, E De March2, M Roussel5, M L Boulland6, R Houot7, A Renault8, T Fest9, G Semenzato2, T Loughran3, T Lamy10. 1. Department of Clinical Hematology, Rennes University Hospital, Rennes, France; Department of Pathology, Rennes University Hospital, Rennes, France. 2. Department of Medicine, Hematology and Clinical Immunology Branch, Padua University School of Medicine, Padua, Italy. 3. University of Virginia Cancer Center, University of Virginia School of Medicine, Charlottesville, USA. 4. Department of Clinical Hematology, Clinique Cesson-Sévigné, Cesson-Sévigné. 5. Department of Hematology-Immunology and Cell Therapy, Rennes University Hospital, Rennes; INSERM UMR 917 Faculté de médecine Université Rennes 1, Rennes. 6. Department of Hematology-Immunology and Cell Therapy, Rennes University Hospital, Rennes. 7. Department of Clinical Hematology, Rennes University Hospital, Rennes, France; INSERM UMR 917 Faculté de médecine Université Rennes 1, Rennes. 8. Department of Clinical Investigation, Rennes University Hospital, Rennes, France. 9. Department of Clinical Hematology, Clinique Cesson-Sévigné, Cesson-Sévigné; INSERM UMR 917 Faculté de médecine Université Rennes 1, Rennes. 10. Department of Clinical Hematology, Rennes University Hospital, Rennes, France; INSERM UMR 917 Faculté de médecine Université Rennes 1, Rennes; Department of Clinical Investigation, Rennes University Hospital, Rennes, France. Electronic address: thierry.lamy@univ-rennes1.fr.
Abstract
BACKGROUND: The 2008 World Health Organization (WHO) classification distinguishes three entities among the large granular lymphocytic leukemia (LGL leukemia): T-cell LGL leukemia (T-LGL leukemia), aggressive natural killer (NK) cell leukemia, and chronic NK lymphoproliferative disorders (LPD), the later considered as a provisional entity. Only a few and small cohorts of chronic NK LPD have been published. PATIENTS AND METHODS: We report here clinicobiological features collected retrospectively from 70 cases of chronic NK LPD, and compared with those of T-LGL leukemia. RESULTS: There were no statistical differences between chronic NK LPD and T-LGL leukemia concerning median age [61 years (range 23-82 years)], organomegaly (26%), associated autoimmune diseases (24%), and associated hematological malignancies (11%). Patients with chronic NK LPD were significantly less symptomatic (49% versus 18%, P < 0.001) and the association with rheumatoid arthritis was more rarely observed (7% versus 17%, P = 0.03). The neutropenia (<0.5 × 10(9)/l) was less severe in chronic NK LPD (33% versus 61%, P < 0.001) without difference in the rate of recurrent infections. STAT3 mutation was detected in 12% of the cohort, which is lower than the frequency observed in T-LGL leukemia. Thirty-seven percent of the patients required specific therapy. Good results were obtained with cyclophosphamide. Overall and complete response rates were, respectively, 69% and 56%. Overall survival was 94% at 5 years. CONCLUSION: This study suggests very high similarities between chronic NK LPD and T-LGL leukemias. Since chronic NK LPD is still a provisional entity, our findings should be helpful when considering further revisions of the WHO classification.
BACKGROUND: The 2008 World Health Organization (WHO) classification distinguishes three entities among the large granular lymphocytic leukemia (LGL leukemia): T-cell LGL leukemia (T-LGL leukemia), aggressive natural killer (NK) cell leukemia, and chronic NK lymphoproliferative disorders (LPD), the later considered as a provisional entity. Only a few and small cohorts of chronic NK LPD have been published. PATIENTS AND METHODS: We report here clinicobiological features collected retrospectively from 70 cases of chronic NK LPD, and compared with those of T-LGL leukemia. RESULTS: There were no statistical differences between chronic NK LPD and T-LGL leukemia concerning median age [61 years (range 23-82 years)], organomegaly (26%), associated autoimmune diseases (24%), and associated hematological malignancies (11%). Patients with chronic NK LPD were significantly less symptomatic (49% versus 18%, P < 0.001) and the association with rheumatoid arthritis was more rarely observed (7% versus 17%, P = 0.03). The neutropenia (<0.5 × 10(9)/l) was less severe in chronic NK LPD (33% versus 61%, P < 0.001) without difference in the rate of recurrent infections. STAT3 mutation was detected in 12% of the cohort, which is lower than the frequency observed in T-LGL leukemia. Thirty-seven percent of the patients required specific therapy. Good results were obtained with cyclophosphamide. Overall and complete response rates were, respectively, 69% and 56%. Overall survival was 94% at 5 years. CONCLUSION: This study suggests very high similarities between chronic NK LPD and T-LGL leukemias. Since chronic NK LPD is still a provisional entity, our findings should be helpful when considering further revisions of the WHO classification.
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