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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Universidad Nacional Córdoba. 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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I10-R327-article-30373 |
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ojs |
| institution |
Universidad Nacional de Córdoba |
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I-10 |
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R-327 |
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Revista de la Facultad de Ciencias Médicas de Córdoba |
| 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 Córdoba. 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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I10-R327-article-303732021-11-18T12:43:09Z Hemophagocytic lymphohistiocytosis as a presentation of T-cell lymphoma: Case report: Hemophagocytic lymphohistiocytosis Linfohistiocitosis hemofagocítica como presentación de Linfoma de células-T: Caso clínico: Linfohistiocitosis hemofagocítica Linfohistiocitose hemofagocítica como uma apresentação de linfoma de células T: Relato de caso: Linfohistiocitose hemofagocítica Casares Diaz, Silvina Ayelén Milanesio, Martín Amelia, Enzo Marcelo Saad, Emanuel José Guanchiale, Luciana Alomar, Juan Manuel lymphohistiocytosis, hemophagocytic lymphoma immunocompromised host lymphadenopathy linfohistiocitosis hemofagocítica linfoma huésped inmunocomprometido linfadenopatía linfo-histiocitose hemofagocítica linfoma hospedeiro imunocomprometido linfadenopatia 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 Introducción: La linfohistiocitosis hemofagocítica (LHH) es un síndrome hiperinflamatorio severo causado por activación aberrante de macrófagos y células T citotóxicas que se manifiesta clínicamente como un cuadro febril asociado a citopenias, hiperferritininemia y esplenomegalia. Ante su diagnóstico en adultos se deben buscar causas secundarias como neoplasias hematológicas. El inicio precoz del tratamiento es importante debido a su elevada mortalidad. Métodos: Se presenta el caso de una paciente de 53 años de edad que se le realizó diagnóstico de LHH asociada a Linfoma de células T. La forma de presentación de dicho cuadro fue de manera abrupta con varios síntomas y signos de dicho síndrome, además de múltiples complicaciones infecciosas asociadas. Resultados: Ante el diagnóstico inicial de LHH se inició tratamiento con etopósido y dexametasona, y al obtener el diagnóstico de Linfoma de células T, se instauró tratamiento quimioterápico con esquema de CHOEP, con evolución inicial favorable. Conclusiones: La LHH es una entidad poco frecuente asociada a una elevada mortalidad, por lo que su índice de sospecha debe de ser elevado ante un cuadro clínico y analítico compatible. Sin embargo, su diagnóstico constituye un gran desafío clínico, pudiendo presentarse en algunas ocasiones superpuesto a cuadros infecciosos múltiples. El inicio precoz del tratamiento es importante debido a su alta mortalidad. Introdução: A linfo-histiocitose hemofagocítica (HHL) é uma síndrome hiperinflamatória grave causada pela ativação aberrante de macrófagos e células T citotóxicas que se manifesta clinicamente como quadro febril associado a citopenias, ferritina elevada e esplenomegalia. Antes de seu diagnóstico em adultas, as causas secundárias, como as neoplasias hematológicas, devem ser investigadas. O início precoce do tratamento é importante devido à sua alta mortalidade. Métodos: Apresentamos o caso de um paciente de 53 anos de idade com diagnóstico de HHL associado a linfoma de células T. A apresentação do quadro foi abrupta com diversos sintomas e sinais da referido síndrome, além de múltiplas complicações infecciosas associadas. Resultados: Antes do diagnóstico inicial de HHL, iniciou-se o tratamento com etoposídeo e dexametasona, e quando o diagnóstico de linfoma de células T foi obtido, iniciou-se o tratamento quimioterápico com esquema CHOEP, com evolução inicial favorável. Conclusões: O HHL é uma entidade rara associada a alta mortalidade, portanto seu índice de suspeita deve ser alto quando se depara com um quadro clínico e analítico compatível. No entanto, seu diagnóstico constitui um grande desafio clínico, podendo, às vezes, aparecer sobreposto a múltiplos quadros infecciosos. O início precoce do tratamento é importante devido à alta mortalidade dessa patologia. Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2021-08-23 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion text/html application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/30373 10.31053/1853.0605.v78.n3.30373 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 78 No. 3 (2021); 317-321 Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 78 Núm. 3 (2021); 317-321 Revista da Faculdade de Ciências Médicas de Córdoba; v. 78 n. 3 (2021); 317-321 1853-0605 0014-6722 10.31053/1853.0605.v78.n3 spa https://revistas.unc.edu.ar/index.php/med/article/view/30373/35197 https://revistas.unc.edu.ar/index.php/med/article/view/30373/35228 Derechos de autor 2021 Universidad Nacional de Córdoba http://creativecommons.org/licenses/by-nc/4.0 |