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Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)

Patients with large granular lymphocytic leukemia (LGLL) frequently present with neutropenia. When present, anemia is usually accompanied by neutropenia and/or thrombocytopenia and isolated anemia is uncommon. We evaluated a cohort of 244 LGLL patients spanning 15 years and herein report the clinico...

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Autores principales: Salama, Youssef, Zhao, Fang, Oliveira, Jennifer L., Yuan, Ji, Jevremovic, Dragan, Go, Ronald S., Ding, Wei, Parikh, Sameer A., Shah, Mithun V., Hampel, Paul J., Al-Kali, Aref, Morice, William G., Shi, Min
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8863822/
https://www.ncbi.nlm.nih.gov/pubmed/35194022
http://dx.doi.org/10.1038/s41408-022-00632-6
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author Salama, Youssef
Zhao, Fang
Oliveira, Jennifer L.
Yuan, Ji
Jevremovic, Dragan
Go, Ronald S.
Ding, Wei
Parikh, Sameer A.
Shah, Mithun V.
Hampel, Paul J.
Al-Kali, Aref
Morice, William G.
Shi, Min
author_facet Salama, Youssef
Zhao, Fang
Oliveira, Jennifer L.
Yuan, Ji
Jevremovic, Dragan
Go, Ronald S.
Ding, Wei
Parikh, Sameer A.
Shah, Mithun V.
Hampel, Paul J.
Al-Kali, Aref
Morice, William G.
Shi, Min
author_sort Salama, Youssef
collection PubMed
description Patients with large granular lymphocytic leukemia (LGLL) frequently present with neutropenia. When present, anemia is usually accompanied by neutropenia and/or thrombocytopenia and isolated anemia is uncommon. We evaluated a cohort of 244 LGLL patients spanning 15 years and herein report the clinicopathologic features of 34 (14%) with isolated anemia. The patients with isolated anemia showed a significantly male predominance (p = 0.001), a lower level of hemoglobulin (p < 0.0001) and higher MCV (p = 0.017) and were less likely to have rheumatoid arthritis (p = 0.023) compared to the remaining 210 patients. Of the 34 LGLL patients with isolated anemia, 13 (38%) presented with pure red cell aplasia (PRCA), markedly decreased reticulocyte count and erythroid precursors, and more transfusion-dependence when compared to non-PRCA patients. There was no other significant clinicopathologic difference between PRCA and non-PRCA patients. 32 patients were followed for a median duration of 51 months (6–199). 24 patients were treated (11/11 PRCA and 13/21 non-PRCA patients, p < 0.02). The overall response rate to first-line therapy was 83% [8/11 (72.7%) for PRCA, 12/13 (92.3%) for non-PRCA], including 14 showing complete response and 6 showing partial response with a median response duration of 48 months (12–129). Half of non-PRCA patients who were observed experienced progressive anemia. During follow-up, no patients developed neutropenia; however, 5/27 (18.5%) patients developed thrombocytopenia. No significant difference in overall survival was noted between PRCA and non-PRCA patients. In summary, this study demonstrates the unique features of LGLL with isolated anemia and underscores the importance of recognizing LGLL as a potential cause of isolated anemia, which may benefit from disease-specific treatment. LGLL patients with PRCA were more likely to require treatment but demonstrated similar clinicopathologic features, therapeutic responses, and overall survival compared to isolated anemia without PRCA, suggesting PRCA and non-PRCA of T-LGLL belong to a common disease spectrum.
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spelling pubmed-88638222022-03-17 Isolated anemia in patients with large granular lymphocytic leukemia (LGLL) Salama, Youssef Zhao, Fang Oliveira, Jennifer L. Yuan, Ji Jevremovic, Dragan Go, Ronald S. Ding, Wei Parikh, Sameer A. Shah, Mithun V. Hampel, Paul J. Al-Kali, Aref Morice, William G. Shi, Min Blood Cancer J Article Patients with large granular lymphocytic leukemia (LGLL) frequently present with neutropenia. When present, anemia is usually accompanied by neutropenia and/or thrombocytopenia and isolated anemia is uncommon. We evaluated a cohort of 244 LGLL patients spanning 15 years and herein report the clinicopathologic features of 34 (14%) with isolated anemia. The patients with isolated anemia showed a significantly male predominance (p = 0.001), a lower level of hemoglobulin (p < 0.0001) and higher MCV (p = 0.017) and were less likely to have rheumatoid arthritis (p = 0.023) compared to the remaining 210 patients. Of the 34 LGLL patients with isolated anemia, 13 (38%) presented with pure red cell aplasia (PRCA), markedly decreased reticulocyte count and erythroid precursors, and more transfusion-dependence when compared to non-PRCA patients. There was no other significant clinicopathologic difference between PRCA and non-PRCA patients. 32 patients were followed for a median duration of 51 months (6–199). 24 patients were treated (11/11 PRCA and 13/21 non-PRCA patients, p < 0.02). The overall response rate to first-line therapy was 83% [8/11 (72.7%) for PRCA, 12/13 (92.3%) for non-PRCA], including 14 showing complete response and 6 showing partial response with a median response duration of 48 months (12–129). Half of non-PRCA patients who were observed experienced progressive anemia. During follow-up, no patients developed neutropenia; however, 5/27 (18.5%) patients developed thrombocytopenia. No significant difference in overall survival was noted between PRCA and non-PRCA patients. In summary, this study demonstrates the unique features of LGLL with isolated anemia and underscores the importance of recognizing LGLL as a potential cause of isolated anemia, which may benefit from disease-specific treatment. LGLL patients with PRCA were more likely to require treatment but demonstrated similar clinicopathologic features, therapeutic responses, and overall survival compared to isolated anemia without PRCA, suggesting PRCA and non-PRCA of T-LGLL belong to a common disease spectrum. Nature Publishing Group UK 2022-02-22 /pmc/articles/PMC8863822/ /pubmed/35194022 http://dx.doi.org/10.1038/s41408-022-00632-6 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Article
Salama, Youssef
Zhao, Fang
Oliveira, Jennifer L.
Yuan, Ji
Jevremovic, Dragan
Go, Ronald S.
Ding, Wei
Parikh, Sameer A.
Shah, Mithun V.
Hampel, Paul J.
Al-Kali, Aref
Morice, William G.
Shi, Min
Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)
title Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)
title_full Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)
title_fullStr Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)
title_full_unstemmed Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)
title_short Isolated anemia in patients with large granular lymphocytic leukemia (LGLL)
title_sort isolated anemia in patients with large granular lymphocytic leukemia (lgll)
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8863822/
https://www.ncbi.nlm.nih.gov/pubmed/35194022
http://dx.doi.org/10.1038/s41408-022-00632-6
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