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Un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d’un cas marocain et revue de la littérature

In the last classification of the World Health Organization (WHO) 2016, T-cell large granular lymphocyte (LGL) leukemia is a type of mature natural killer T (NKT) cell lymphatic hematologic disease. T-LGL leukemia is characterized by CD3+ T-cell phenotype and TCR gene rearrangement (T lymphocyte rec...

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Autores principales: Benmoussa, Amine, Oussaih, Leila, Tazi, Illias
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The African Field Epidemiology Network 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6945371/
https://www.ncbi.nlm.nih.gov/pubmed/31934260
http://dx.doi.org/10.11604/pamj.2019.34.119.19715
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author Benmoussa, Amine
Oussaih, Leila
Tazi, Illias
author_facet Benmoussa, Amine
Oussaih, Leila
Tazi, Illias
author_sort Benmoussa, Amine
collection PubMed
description In the last classification of the World Health Organization (WHO) 2016, T-cell large granular lymphocyte (LGL) leukemia is a type of mature natural killer T (NKT) cell lymphatic hematologic disease. T-LGL leukemia is characterized by CD3+ T-cell phenotype and TCR gene rearrangement (T lymphocyte receptor). It is a rare disease, mainly affecting people living in the Western world. We report the case of a 60-year old Moroccan patient presenting with 1-month history of generalized jaundice with diffuse petechiae and impaired general condition. Initial clinical examination showed ascites of average abundance and hepatosplenomegaly; paraclinical examination objectified hepatocellular failure with neutropenia and thrombocytopenia. The diagnosis of T-LGL was retained based on cytological and immunophenotypic study of bone marrow blood as well as on the detection of TCR rearrangement using molecular test. The patient received cyclophosphamide associated with symptomatic treatment. The patient died within three months of the diagnosis due to septic shock with cachexia. T-LGL often has an indolent behavior and initial clinical status at diagnosis is generally moderate, with progressive onset but that was not so for our patient. Hepatocellular failure exceptionally results in T-cell large granular lymphocyte (LGL) leukemia.
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spelling pubmed-69453712020-01-13 Un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d’un cas marocain et revue de la littérature Benmoussa, Amine Oussaih, Leila Tazi, Illias Pan Afr Med J Case Report In the last classification of the World Health Organization (WHO) 2016, T-cell large granular lymphocyte (LGL) leukemia is a type of mature natural killer T (NKT) cell lymphatic hematologic disease. T-LGL leukemia is characterized by CD3+ T-cell phenotype and TCR gene rearrangement (T lymphocyte receptor). It is a rare disease, mainly affecting people living in the Western world. We report the case of a 60-year old Moroccan patient presenting with 1-month history of generalized jaundice with diffuse petechiae and impaired general condition. Initial clinical examination showed ascites of average abundance and hepatosplenomegaly; paraclinical examination objectified hepatocellular failure with neutropenia and thrombocytopenia. The diagnosis of T-LGL was retained based on cytological and immunophenotypic study of bone marrow blood as well as on the detection of TCR rearrangement using molecular test. The patient received cyclophosphamide associated with symptomatic treatment. The patient died within three months of the diagnosis due to septic shock with cachexia. T-LGL often has an indolent behavior and initial clinical status at diagnosis is generally moderate, with progressive onset but that was not so for our patient. Hepatocellular failure exceptionally results in T-cell large granular lymphocyte (LGL) leukemia. The African Field Epidemiology Network 2019-10-29 /pmc/articles/PMC6945371/ /pubmed/31934260 http://dx.doi.org/10.11604/pamj.2019.34.119.19715 Text en © Amine Benmoussa et al. http://creativecommons.org/licenses/by/2.0/ The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Benmoussa, Amine
Oussaih, Leila
Tazi, Illias
Un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d’un cas marocain et revue de la littérature
title Un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d’un cas marocain et revue de la littérature
title_full Un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d’un cas marocain et revue de la littérature
title_fullStr Un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d’un cas marocain et revue de la littérature
title_full_unstemmed Un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d’un cas marocain et revue de la littérature
title_short Un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type T: à propos d’un cas marocain et revue de la littérature
title_sort un syndrome d’insuffisance hépatocellulaire révélant une leucémie à grands lymphocytes granuleux type t: à propos d’un cas marocain et revue de la littérature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6945371/
https://www.ncbi.nlm.nih.gov/pubmed/31934260
http://dx.doi.org/10.11604/pamj.2019.34.119.19715
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