Cargando…

Syndrome d’activation macrophagique révélant un lymphome T sous-cutané chez un adolescent de 16 ans

Macrophage activation syndrome (MAS) is a multisystemic disorder resulting from an over-activation of the immune system leading to a more or less diffuse macrophagic infiltration into the tussues. Clinical signs including fever, hepatosplenomegaly, adenopathy are associated with abnormal values in l...

Descripción completa

Detalles Bibliográficos
Autores principales: Hbibi, Mohamed, Benmiloud, Sara, Rahmouni, Safae, Tadmouri, Ilhame, Abourazzak, Sana, Chaouki, Sana, Souilmi, Fatima Zahra, Idrissi, Mounia Lakhdar, Hida, Mostapha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The African Field Epidemiology Network 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6457733/
https://www.ncbi.nlm.nih.gov/pubmed/31007821
http://dx.doi.org/10.11604/pamj.2018.31.74.14686
_version_ 1783409934993457152
author Hbibi, Mohamed
Benmiloud, Sara
Rahmouni, Safae
Tadmouri, Ilhame
Abourazzak, Sana
Chaouki, Sana
Souilmi, Fatima Zahra
Idrissi, Mounia Lakhdar
Hida, Mostapha
author_facet Hbibi, Mohamed
Benmiloud, Sara
Rahmouni, Safae
Tadmouri, Ilhame
Abourazzak, Sana
Chaouki, Sana
Souilmi, Fatima Zahra
Idrissi, Mounia Lakhdar
Hida, Mostapha
author_sort Hbibi, Mohamed
collection PubMed
description Macrophage activation syndrome (MAS) is a multisystemic disorder resulting from an over-activation of the immune system leading to a more or less diffuse macrophagic infiltration into the tussues. Clinical signs including fever, hepatosplenomegaly, adenopathy are associated with abnormal values in laboratory test results (bi or pancytopenia, hepatic cytolysis, elevated LDH levels, coagulopathy) and hemophagocytosis. In children, it can be primary or secondary to several disorders. We report the case of a 16-year old patient admitted to the Department of Pediatrics with febrile leukopenia associated with impaired general condition and circular erythematous lesions spread to the lower limbs. The diagnosis of macrophage activation syndrome was retained based on clinical signs, laboratory test results and cytological results. Skin biopsy of these lesions showed subcutaneous panniculitis-like T-cell lymphoma. The purpose of this study is to insists on the peculiarities of this clinical case given the rarity of this type of subcutaneous T-cell lymphoma, much more in this age group. We want to highlight the importance of suspecting subcutaneous T-cell lymphoma in patients with MAS associated with subcutaneous erythematous lesions because this could be life threatening.
format Online
Article
Text
id pubmed-6457733
institution National Center for Biotechnology Information
language English
publishDate 2018
publisher The African Field Epidemiology Network
record_format MEDLINE/PubMed
spelling pubmed-64577332019-04-19 Syndrome d’activation macrophagique révélant un lymphome T sous-cutané chez un adolescent de 16 ans Hbibi, Mohamed Benmiloud, Sara Rahmouni, Safae Tadmouri, Ilhame Abourazzak, Sana Chaouki, Sana Souilmi, Fatima Zahra Idrissi, Mounia Lakhdar Hida, Mostapha Pan Afr Med J Case Report Macrophage activation syndrome (MAS) is a multisystemic disorder resulting from an over-activation of the immune system leading to a more or less diffuse macrophagic infiltration into the tussues. Clinical signs including fever, hepatosplenomegaly, adenopathy are associated with abnormal values in laboratory test results (bi or pancytopenia, hepatic cytolysis, elevated LDH levels, coagulopathy) and hemophagocytosis. In children, it can be primary or secondary to several disorders. We report the case of a 16-year old patient admitted to the Department of Pediatrics with febrile leukopenia associated with impaired general condition and circular erythematous lesions spread to the lower limbs. The diagnosis of macrophage activation syndrome was retained based on clinical signs, laboratory test results and cytological results. Skin biopsy of these lesions showed subcutaneous panniculitis-like T-cell lymphoma. The purpose of this study is to insists on the peculiarities of this clinical case given the rarity of this type of subcutaneous T-cell lymphoma, much more in this age group. We want to highlight the importance of suspecting subcutaneous T-cell lymphoma in patients with MAS associated with subcutaneous erythematous lesions because this could be life threatening. The African Field Epidemiology Network 2018-10-02 /pmc/articles/PMC6457733/ /pubmed/31007821 http://dx.doi.org/10.11604/pamj.2018.31.74.14686 Text en © Mohamed Hbibi 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
Hbibi, Mohamed
Benmiloud, Sara
Rahmouni, Safae
Tadmouri, Ilhame
Abourazzak, Sana
Chaouki, Sana
Souilmi, Fatima Zahra
Idrissi, Mounia Lakhdar
Hida, Mostapha
Syndrome d’activation macrophagique révélant un lymphome T sous-cutané chez un adolescent de 16 ans
title Syndrome d’activation macrophagique révélant un lymphome T sous-cutané chez un adolescent de 16 ans
title_full Syndrome d’activation macrophagique révélant un lymphome T sous-cutané chez un adolescent de 16 ans
title_fullStr Syndrome d’activation macrophagique révélant un lymphome T sous-cutané chez un adolescent de 16 ans
title_full_unstemmed Syndrome d’activation macrophagique révélant un lymphome T sous-cutané chez un adolescent de 16 ans
title_short Syndrome d’activation macrophagique révélant un lymphome T sous-cutané chez un adolescent de 16 ans
title_sort syndrome d’activation macrophagique révélant un lymphome t sous-cutané chez un adolescent de 16 ans
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6457733/
https://www.ncbi.nlm.nih.gov/pubmed/31007821
http://dx.doi.org/10.11604/pamj.2018.31.74.14686
work_keys_str_mv AT hbibimohamed syndromedactivationmacrophagiquerevelantunlymphometsouscutanechezunadolescentde16ans
AT benmiloudsara syndromedactivationmacrophagiquerevelantunlymphometsouscutanechezunadolescentde16ans
AT rahmounisafae syndromedactivationmacrophagiquerevelantunlymphometsouscutanechezunadolescentde16ans
AT tadmouriilhame syndromedactivationmacrophagiquerevelantunlymphometsouscutanechezunadolescentde16ans
AT abourazzaksana syndromedactivationmacrophagiquerevelantunlymphometsouscutanechezunadolescentde16ans
AT chaoukisana syndromedactivationmacrophagiquerevelantunlymphometsouscutanechezunadolescentde16ans
AT souilmifatimazahra syndromedactivationmacrophagiquerevelantunlymphometsouscutanechezunadolescentde16ans
AT idrissimounialakhdar syndromedactivationmacrophagiquerevelantunlymphometsouscutanechezunadolescentde16ans
AT hidamostapha syndromedactivationmacrophagiquerevelantunlymphometsouscutanechezunadolescentde16ans