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Large Granular Lymphocytes Leukemia: A Case Report with a Review of the Literature

Received: 6 December 2019    Accepted: 26 December 2019    Published: 9 January 2020
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Abstract

Larges granulars lymphocytes (LGL) leukemias correspond to rare haemopathies secondary to clonal proliferation of larges lymphocytes rich in azurophilic granules with phenotype T of CD3+ or CD3- (NK cell) profile. We report the case of a patient, 75 years old, who was admitted for pancytopenia associated with lymphocytosis at 8G / L all evolving in a febrile context. The clinical examination did not find tumoral syndrome. The blood smear founded very numerous granulars lymphocytes representing 46% of all lymphocytes. The myelogram shows an aspect in favor of peripheral thrombocytopenia without other notable abnormalities. There is a cellular expansion of CD8+ NK T cells compatible with LGL-type proliferation. The bone marrow is infiltrated by these lymphocytes with a percentage of 28%. It is a pathology with an heterogeneous spectrum classified by the World Health Organization into three entities: LGL T leukemia and chronic NK cell expansions with chronic and indolent evolution and aggressive cell leukemia NK with usually a reserved prognosis. Recent advances in the knowledge of the pathophysiology of these haemopathies have made it possible to specify the mechanisms underlying the perpetuation of the LGL clone and to identify new therapeutic targets.

Published in American Journal of Biomedical and Life Sciences (Volume 8, Issue 1)
DOI 10.11648/j.ajbls.20200801.11
Page(s) 1-4
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Large Granular Lymphocytes Leukemias, Case Report, Three Entities, Literature Review

References
[1] Lazaro E, Duffau P, Chaigne, Delalande S, Greib C, Pellegrin J-L et al. Les leucémies à grands lymphocytes granuleux: de la clinique à la physiopathologie. La revue de med int 2013; 34: 553-560.
[2] Semenzato G, Pandolfi F, Chisesi T, De Rossi G, Pizzolo G, Zambello R et al. The lymphoproliferative disease of granular lymphocytes. A heterogeneous disorder ranging from indolent to agressive conditions. Cancer 1987; 60: 2971-8.
[3] Loughran TP. Clonal diseases of large granural lymphocytes. Blood 1993; 82: 1-14.
[4] Bareau B, Rey J, Hamidou M, Donadieu J, Morcet J, Reman O et al. Analysis of a french cohort of patients with large granular lymphocyte leukemia: a report on 229 cases. Haematologica 2010; 95: 1534-41.
[5] Oshimi K. Progress in understanding and managing naturel killer-cell malignancies. Br J Haematol 2007; 139: 532–44.
[6] Pandolfi F, Loughran TP, Starkebaum G, Chisesi T, Barbui T, Chan WC, et al. Clinical course and prognosis of the lymphoproliferative disease of granular lymphocyte. Amulticer study. Cancer 1990; 65: 341-8.
[7] Benmoussa A, Oussaih L, Tazi I. Un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d’un cas marocain et revue de lalittérature. The Pan African Medical Journal. 2019; 34: 119.
[8] William G, Finn MD, William R, Macon MD. 13-Mature T-Cell and Natural Killer–Cell Leukemias. Hematopathology (third edition) 2018; 414-428.
[9] Dearden C. Large granular lymphocytic leukemia pathogenesis and managment. Br J Haematol2011; 152: 273–83.
[10] Olteanu H, Karandikar NJ, Eshoa C, Kroft SH. Laboratory finding in CD4 (+) large granular lymphocytose. Int J Lab Hematol 2010; 32: e9–16.
[11] Alekshun TJ, Tao J, Sokol L. Agressive T-cell large granular lymphocyte leukemia: a case report and review of the litterature. Am J Hematol 2007; 82: 481-5.
[12] Feng B, Jorgensen JL, Hu Y, Medeiros LJ, Wang SA. TCR-Vbeta flow cytometric analysis of peripheral blood for assessing clonality and desease burden in patients with T-cell large granular lymphocyte leukemia. J Clin Pathol 2010; 63: 141–6.
[13] Gattazo C, Teramo A, Miorin M, Squizzato E, Cabrelle A, Balsamo M ? et al. Lack of expression of inhibitory KIR3DL1 receptor in patients with natural killer cell-type lymphoproliferative disease of granular lymphocytes. Haematologica 2010; 95: 1722-9.
[14] Wong KF, Chan JC, Liu HS, Man C, Kwong KL. Chromosomal abnormalities in T-cell large granular lymphocyte leukemia: report of two cases and review of the litterature. Br J Haematol 2002; 116: 598–600.
[15] B. Moosica K, Pailad U, C. Olsona K, Dziewulskaa K, Wang T, Xing JC, et al. Genomics of LGL leukemia and select other rare leukemia/ lymphomas. Best Practice & Research Clinical Haematology 2019; 32: 196-206.
[16] Lamy T, Loughran TP. How i treat LGL leukemia? Blood 2011; 117: 2764-74.
[17] Suziki R, Suzumiya R, Nakamura S, Aoki S, Notoya A, Ozaki S, et al. Agressive natural killer-cell leukemia revisited; large granular lymphocyte leukemia of cytotoxic NK cells. Leukemia 2004; 18: 763-70.
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  • APA Style

    Maryam Mouamin, Ghita Badou, Hicham Yahyaoui, Imane Benjelloun, Mohamed Raissi, et al. (2020). Large Granular Lymphocytes Leukemia: A Case Report with a Review of the Literature. American Journal of Biomedical and Life Sciences, 8(1), 1-4. https://doi.org/10.11648/j.ajbls.20200801.11

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    ACS Style

    Maryam Mouamin; Ghita Badou; Hicham Yahyaoui; Imane Benjelloun; Mohamed Raissi, et al. Large Granular Lymphocytes Leukemia: A Case Report with a Review of the Literature. Am. J. Biomed. Life Sci. 2020, 8(1), 1-4. doi: 10.11648/j.ajbls.20200801.11

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    AMA Style

    Maryam Mouamin, Ghita Badou, Hicham Yahyaoui, Imane Benjelloun, Mohamed Raissi, et al. Large Granular Lymphocytes Leukemia: A Case Report with a Review of the Literature. Am J Biomed Life Sci. 2020;8(1):1-4. doi: 10.11648/j.ajbls.20200801.11

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  • @article{10.11648/j.ajbls.20200801.11,
      author = {Maryam Mouamin and Ghita Badou and Hicham Yahyaoui and Imane Benjelloun and Mohamed Raissi and Mustapha Ait Ameur and Mohamed Chakour},
      title = {Large Granular Lymphocytes Leukemia: A Case Report with a Review of the Literature},
      journal = {American Journal of Biomedical and Life Sciences},
      volume = {8},
      number = {1},
      pages = {1-4},
      doi = {10.11648/j.ajbls.20200801.11},
      url = {https://doi.org/10.11648/j.ajbls.20200801.11},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajbls.20200801.11},
      abstract = {Larges granulars lymphocytes (LGL) leukemias correspond to rare haemopathies secondary to clonal proliferation of larges lymphocytes rich in azurophilic granules with phenotype T of CD3+ or CD3- (NK cell) profile. We report the case of a patient, 75 years old, who was admitted for pancytopenia associated with lymphocytosis at 8G / L all evolving in a febrile context. The clinical examination did not find tumoral syndrome. The blood smear founded very numerous granulars lymphocytes representing 46% of all lymphocytes. The myelogram shows an aspect in favor of peripheral thrombocytopenia without other notable abnormalities. There is a cellular expansion of CD8+ NK T cells compatible with LGL-type proliferation. The bone marrow is infiltrated by these lymphocytes with a percentage of 28%. It is a pathology with an heterogeneous spectrum classified by the World Health Organization into three entities: LGL T leukemia and chronic NK cell expansions with chronic and indolent evolution and aggressive cell leukemia NK with usually a reserved prognosis. Recent advances in the knowledge of the pathophysiology of these haemopathies have made it possible to specify the mechanisms underlying the perpetuation of the LGL clone and to identify new therapeutic targets.},
     year = {2020}
    }
    

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    AB  - Larges granulars lymphocytes (LGL) leukemias correspond to rare haemopathies secondary to clonal proliferation of larges lymphocytes rich in azurophilic granules with phenotype T of CD3+ or CD3- (NK cell) profile. We report the case of a patient, 75 years old, who was admitted for pancytopenia associated with lymphocytosis at 8G / L all evolving in a febrile context. The clinical examination did not find tumoral syndrome. The blood smear founded very numerous granulars lymphocytes representing 46% of all lymphocytes. The myelogram shows an aspect in favor of peripheral thrombocytopenia without other notable abnormalities. There is a cellular expansion of CD8+ NK T cells compatible with LGL-type proliferation. The bone marrow is infiltrated by these lymphocytes with a percentage of 28%. It is a pathology with an heterogeneous spectrum classified by the World Health Organization into three entities: LGL T leukemia and chronic NK cell expansions with chronic and indolent evolution and aggressive cell leukemia NK with usually a reserved prognosis. Recent advances in the knowledge of the pathophysiology of these haemopathies have made it possible to specify the mechanisms underlying the perpetuation of the LGL clone and to identify new therapeutic targets.
    VL  - 8
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Author Information
  • Hematology Laboratory of Avicene Military Hospital, Faculty of Medicine and Pharmacy, Qadi Iyyad University, Marrakech, Morrocco

  • Hematology Laboratory of Avicene Military Hospital, Faculty of Medicine and Pharmacy, Qadi Iyyad University, Marrakech, Morrocco

  • Hematology Laboratory of Avicene Military Hospital, Faculty of Medicine and Pharmacy, Qadi Iyyad University, Marrakech, Morrocco

  • Hematology Laboratory of Avicene Military Hospital, Faculty of Medicine and Pharmacy, Qadi Iyyad University, Marrakech, Morrocco

  • Hematology Department of Avicene Military Hospital, Faculty of Medicine and Pharmacy, Qadi Iyyad University, Marrakech, Morrocco

  • Hematology Laboratory of Avicene Military Hospital, Faculty of Medicine and Pharmacy, Qadi Iyyad University, Marrakech, Morrocco

  • Hematology Laboratory of Avicene Military Hospital, Faculty of Medicine and Pharmacy, Qadi Iyyad University, Marrakech, Morrocco

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