Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis
Introduction: Hemophagocytic lymphohistiocytosis (HHL), a severe hyperinflammatory syndrome caused by aberrant activation of macrophages and cytotoxic T cells, is clinically manifested as a febrile onset along with cytopenias, high ferritin serum level and splenomegaly. In adult patients, secondary c...
Guardado en:
Autores principales: | , , , , , |
---|---|
Formato: | Artículo revista |
Lenguaje: | Español |
Publicado: |
Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2021
|
Materias: | |
Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/30373 |
Aporte de: |
id |
I10-R10article-30373 |
---|---|
record_format |
ojs |
institution |
Universidad Nacional de Córdoba |
institution_str |
I-10 |
repository_str |
R-10 |
container_title_str |
Revistas de la UNC |
language |
Español |
format |
Artículo revista |
topic |
lymphohistiocytosis, hemophagocytic lymphoma immunocompromised host lymphadenopathy linfohistiocitosis hemofagocítica linfoma huésped inmunocomprometido linfadenopatía linfo-histiocitose hemofagocítica linfoma hospedeiro imunocomprometido linfadenopatia |
spellingShingle |
lymphohistiocytosis, hemophagocytic lymphoma immunocompromised host lymphadenopathy linfohistiocitosis hemofagocítica linfoma huésped inmunocomprometido linfadenopatía linfo-histiocitose hemofagocítica linfoma hospedeiro imunocomprometido linfadenopatia Casares Diaz, Silvina Ayelén Milanesio, Martín Amelia, Enzo Marcelo Saad, Emanuel José Guanchiale, Luciana Alomar, Juan Manuel Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis |
topic_facet |
lymphohistiocytosis, hemophagocytic lymphoma immunocompromised host lymphadenopathy linfohistiocitosis hemofagocítica linfoma huésped inmunocomprometido linfadenopatía linfo-histiocitose hemofagocítica linfoma hospedeiro imunocomprometido linfadenopatia |
author |
Casares Diaz, Silvina Ayelén Milanesio, Martín Amelia, Enzo Marcelo Saad, Emanuel José Guanchiale, Luciana Alomar, Juan Manuel |
author_facet |
Casares Diaz, Silvina Ayelén Milanesio, Martín Amelia, Enzo Marcelo Saad, Emanuel José Guanchiale, Luciana Alomar, Juan Manuel |
author_sort |
Casares Diaz, Silvina Ayelén |
title |
Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis |
title_short |
Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis |
title_full |
Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis |
title_fullStr |
Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis |
title_full_unstemmed |
Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis |
title_sort |
hemophagocytic lymphohistiocytosis as a presentation of t-cell lymphoma: case report: hemophagocytic lymphohistiocytosis |
description |
Introduction: Hemophagocytic lymphohistiocytosis (HHL), a severe hyperinflammatory syndrome caused by aberrant activation of macrophages and cytotoxic T cells, is clinically manifested as a febrile onset along with cytopenias, high ferritin serum level and splenomegaly. In adult patients, secondary causes of it should be looked for, such as autoimmune and infectious diseases and neoplasms. Prompt initiation of treatment is important due to the high mortality of this syndrome.
Methods: A case of a 53-year-old patient diagnosed with HHL associated with T-cell lymphoma is presented as a sudden onset of several symptoms and signs of HHL, along with infectious complications.
Results: Once the diagnosis of HHL was made, treatment with Etoposide and Dexamethasone was started, and with the diagnosis of T-cell lymphoma established, chemotherapy treatment with a CHOEP scheme was started, with a favorable initial evolution.
Main conclusion: As HHL is a rare entity associated with high mortality, initial suspicion must be high when facing an acute onset of fever, cytopenia and splenomegaly. Nonetheless, diagnosing HHL is challenging and often appear superimposed on multiple infectious diseases. The early initiation of treatment is important given the high mortality of this pathology
Key words: lymphohistiocytosis, hemophagocytic; lymphoma; immunocompromised host; lymphadenopathy |
publisher |
Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
publishDate |
2021 |
url |
https://revistas.unc.edu.ar/index.php/med/article/view/30373 |
work_keys_str_mv |
AT casaresdiazsilvinaayelen hemophagocyticlymphohistiocytosisasapresentationoftcelllymphomacasereporthemophagocyticlymphohistiocytosis AT milanesiomartin hemophagocyticlymphohistiocytosisasapresentationoftcelllymphomacasereporthemophagocyticlymphohistiocytosis AT ameliaenzomarcelo hemophagocyticlymphohistiocytosisasapresentationoftcelllymphomacasereporthemophagocyticlymphohistiocytosis AT saademanueljose hemophagocyticlymphohistiocytosisasapresentationoftcelllymphomacasereporthemophagocyticlymphohistiocytosis AT guanchialeluciana hemophagocyticlymphohistiocytosisasapresentationoftcelllymphomacasereporthemophagocyticlymphohistiocytosis AT alomarjuanmanuel hemophagocyticlymphohistiocytosisasapresentationoftcelllymphomacasereporthemophagocyticlymphohistiocytosis AT casaresdiazsilvinaayelen linfohistiocitosishemofagociticacomopresentaciondelinfomadecelulastcasoclinicolinfohistiocitosishemofagocitica AT milanesiomartin linfohistiocitosishemofagociticacomopresentaciondelinfomadecelulastcasoclinicolinfohistiocitosishemofagocitica AT ameliaenzomarcelo linfohistiocitosishemofagociticacomopresentaciondelinfomadecelulastcasoclinicolinfohistiocitosishemofagocitica AT saademanueljose linfohistiocitosishemofagociticacomopresentaciondelinfomadecelulastcasoclinicolinfohistiocitosishemofagocitica AT guanchialeluciana linfohistiocitosishemofagociticacomopresentaciondelinfomadecelulastcasoclinicolinfohistiocitosishemofagocitica AT alomarjuanmanuel linfohistiocitosishemofagociticacomopresentaciondelinfomadecelulastcasoclinicolinfohistiocitosishemofagocitica AT casaresdiazsilvinaayelen linfohistiocitosehemofagociticacomoumaapresentacaodelinfomadecelulastrelatodecasolinfohistiocitosehemofagocitica AT milanesiomartin linfohistiocitosehemofagociticacomoumaapresentacaodelinfomadecelulastrelatodecasolinfohistiocitosehemofagocitica AT ameliaenzomarcelo linfohistiocitosehemofagociticacomoumaapresentacaodelinfomadecelulastrelatodecasolinfohistiocitosehemofagocitica AT saademanueljose linfohistiocitosehemofagociticacomoumaapresentacaodelinfomadecelulastrelatodecasolinfohistiocitosehemofagocitica AT guanchialeluciana linfohistiocitosehemofagociticacomoumaapresentacaodelinfomadecelulastrelatodecasolinfohistiocitosehemofagocitica AT alomarjuanmanuel linfohistiocitosehemofagociticacomoumaapresentacaodelinfomadecelulastrelatodecasolinfohistiocitosehemofagocitica |
bdutipo_str |
Revistas |
_version_ |
1764819783325319170 |