Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínico

Toxic epidermal necrolysis (TEN) is a lethal entity, characterized by extensive epidermal necrosis and multiorgan failure. Hemophagocytic syndrome (HFS) is also a rare and lethal syndrome characterized by hyperinflammation that leads to the appearance of fever, pancytopenia, organomegaly and hemopha...

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Autores principales: Peña,Camila, Voisin,Javier, González,José Tomás, Villegas,Pablo
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2018
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000400523
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spelling oai:scielo:S0034-988720180004005232018-07-03Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínicoPeña,CamilaVoisin,JavierGonzález,José TomásVillegas,Pablo Hodgkin Disease Lymphohistiocytosis Hemophagocytic Stevesns-Johnson Syndrome Toxic epidermal necrolysis (TEN) is a lethal entity, characterized by extensive epidermal necrosis and multiorgan failure. Hemophagocytic syndrome (HFS) is also a rare and lethal syndrome characterized by hyperinflammation that leads to the appearance of fever, pancytopenia, organomegaly and hemophagocytosis. The concomitance of these diseases is extremely uncommon. We report a 38 years old female, who during the course of a HFS secondary to Hodgkin Lymphoma (HL), presented a TEN secondary to antibiotics. She was admitted due to a consumptive syndrome, lymphadenopathy, visceromegaly and severe pancytopenia. Laboratory and bone marrow tests confirmed HFS. Due to constant fever, imipenem was indicated. On the third day she started with pain and skin rash. She evolved with positive Nikolsky sign. Cutaneous biopsy was concordant with extensive TEN, which was managed with intravenous immunoglobulin and dexamethasone. A complete response and normalization of the blood count were achieved. Finally, the lymph node biopsy showed HL of mixed cellularity type, which was managed with 8 cycles of ABVD chemotherapy, achieving complete remission.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.146 n.4 20182018-04-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000400523es10.4067/s0034-98872018000400523
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic Hodgkin Disease
Lymphohistiocytosis
Hemophagocytic
Stevesns-Johnson Syndrome
spellingShingle Hodgkin Disease
Lymphohistiocytosis
Hemophagocytic
Stevesns-Johnson Syndrome
Peña,Camila
Voisin,Javier
González,José Tomás
Villegas,Pablo
Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínico
description Toxic epidermal necrolysis (TEN) is a lethal entity, characterized by extensive epidermal necrosis and multiorgan failure. Hemophagocytic syndrome (HFS) is also a rare and lethal syndrome characterized by hyperinflammation that leads to the appearance of fever, pancytopenia, organomegaly and hemophagocytosis. The concomitance of these diseases is extremely uncommon. We report a 38 years old female, who during the course of a HFS secondary to Hodgkin Lymphoma (HL), presented a TEN secondary to antibiotics. She was admitted due to a consumptive syndrome, lymphadenopathy, visceromegaly and severe pancytopenia. Laboratory and bone marrow tests confirmed HFS. Due to constant fever, imipenem was indicated. On the third day she started with pain and skin rash. She evolved with positive Nikolsky sign. Cutaneous biopsy was concordant with extensive TEN, which was managed with intravenous immunoglobulin and dexamethasone. A complete response and normalization of the blood count were achieved. Finally, the lymph node biopsy showed HL of mixed cellularity type, which was managed with 8 cycles of ABVD chemotherapy, achieving complete remission.
author Peña,Camila
Voisin,Javier
González,José Tomás
Villegas,Pablo
author_facet Peña,Camila
Voisin,Javier
González,José Tomás
Villegas,Pablo
author_sort Peña,Camila
title Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínico
title_short Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínico
title_full Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínico
title_fullStr Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínico
title_full_unstemmed Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin. Caso clínico
title_sort necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de hodgkin. caso clínico
publisher Sociedad Médica de Santiago
publishDate 2018
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018000400523
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