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...

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Autores principales: Casares Diaz, Silvina Ayelén, Milanesio, Martín, Amelia, Enzo Marcelo, Saad, Emanuel José, Guanchiale, Luciana, Alomar, Juan Manuel
Formato: Artículo revista
Lenguaje:Español
Publicado: Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2021
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Acceso en línea:https://revistas.unc.edu.ar/index.php/med/article/view/30373
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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
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