Hemophagocytic lymphohistiocytosis in adults: An under recognized entity

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need...

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Autores principales: Shah, Abdul Rashid, Muzzafar, Tariq, Assi, Rita, Schellingerhout, Dawid, Estrov, Zeev, Tamamyan, Gevorg, Kantarjian, Hagop, Daver, Naval
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2016
Materias:
Regular Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5219626/
https://www.ncbi.nlm.nih.gov/pubmed/28070498
http://dx.doi.org/10.1016/j.bbacli.2016.12.002
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author Shah, Abdul Rashid
Muzzafar, Tariq
Assi, Rita
Schellingerhout, Dawid
Estrov, Zeev
Tamamyan, Gevorg
Kantarjian, Hagop
Daver, Naval
author_facet Shah, Abdul Rashid
Muzzafar, Tariq
Assi, Rita
Schellingerhout, Dawid
Estrov, Zeev
Tamamyan, Gevorg
Kantarjian, Hagop
Daver, Naval
author_sort Shah, Abdul Rashid
collection PubMed
description Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity.
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spelling pubmed-52196262017-01-09 Hemophagocytic lymphohistiocytosis in adults: An under recognized entity Shah, Abdul Rashid Muzzafar, Tariq Assi, Rita Schellingerhout, Dawid Estrov, Zeev Tamamyan, Gevorg Kantarjian, Hagop Daver, Naval BBA Clin Regular Article Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity. Elsevier 2016-12-20 /pmc/articles/PMC5219626/ /pubmed/28070498 http://dx.doi.org/10.1016/j.bbacli.2016.12.002 Text en © 2016 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Regular Article
Shah, Abdul Rashid
Muzzafar, Tariq
Assi, Rita
Schellingerhout, Dawid
Estrov, Zeev
Tamamyan, Gevorg
Kantarjian, Hagop
Daver, Naval
Hemophagocytic lymphohistiocytosis in adults: An under recognized entity
title Hemophagocytic lymphohistiocytosis in adults: An under recognized entity
title_full Hemophagocytic lymphohistiocytosis in adults: An under recognized entity
title_fullStr Hemophagocytic lymphohistiocytosis in adults: An under recognized entity
title_full_unstemmed Hemophagocytic lymphohistiocytosis in adults: An under recognized entity
title_short Hemophagocytic lymphohistiocytosis in adults: An under recognized entity
title_sort hemophagocytic lymphohistiocytosis in adults: an under recognized entity
topic Regular Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5219626/
https://www.ncbi.nlm.nih.gov/pubmed/28070498
http://dx.doi.org/10.1016/j.bbacli.2016.12.002
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