Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system

Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system

The syndrome identified as hemophagocytic lymphohistiocytosis (HLH) poses a rather difficult challenge to the physician. HLH was initially described as a familial disorder,1 which was later associated with various genetic defects that affect the cytotoxic machinery of CD8+ T and NK cells,2 but it a...

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Main Author: José Alexandre Marzagão Barbuto
Format: Article
Language:English
Published: University of São Paulo 2015-03-01
Series:Autopsy and Case Reports
Online Access:http://www.revistas.usp.br/autopsy/article/view/98449
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spelling doaj-03b3262a591c40d2b5090efa645b39292020-11-24T21:46:26ZengUniversity of São PauloAutopsy and Case Reports2236-19602015-03-015110.4322/acr.%y.9844988467Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune systemJosé Alexandre Marzagão Barbuto0Department of Immunology, Institute of Biomedical Sciences; Discipline of Molecular Medicine, Department of Internal Medicine, Universidade de São Paulo, São Paulo/SP The syndrome identified as hemophagocytic lymphohistiocytosis (HLH) poses a rather difficult challenge to the physician. HLH was initially described as a familial disorder,1 which was later associated with various genetic defects that affect the cytotoxic machinery of CD8+ T and NK cells,2 but it also occurs sporadically, usually prompted by infections, rheumatic disorders, or neoplasia.3 Although its diagnostic criteria are established, its clinical presentation overlaps with many different conditions, requiring an enhanced awareness of the attending physician in order to reach the diagnosis and to initiate treatment early enough. http://www.revistas.usp.br/autopsy/article/view/98449
collection DOAJ
language English
format Article
sources DOAJ
author José Alexandre Marzagão Barbuto
spellingShingle José Alexandre Marzagão Barbuto
Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system
Autopsy and Case Reports
author_facet José Alexandre Marzagão Barbuto
author_sort José Alexandre Marzagão Barbuto
title Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system
title_short Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system
title_full Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system
title_fullStr Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system
title_full_unstemmed Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system
title_sort hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system
publisher University of São Paulo
series Autopsy and Case Reports
issn 2236-1960
publishDate 2015-03-01
description The syndrome identified as hemophagocytic lymphohistiocytosis (HLH) poses a rather difficult challenge to the physician. HLH was initially described as a familial disorder,1 which was later associated with various genetic defects that affect the cytotoxic machinery of CD8+ T and NK cells,2 but it also occurs sporadically, usually prompted by infections, rheumatic disorders, or neoplasia.3 Although its diagnostic criteria are established, its clinical presentation overlaps with many different conditions, requiring an enhanced awareness of the attending physician in order to reach the diagnosis and to initiate treatment early enough.
url http://www.revistas.usp.br/autopsy/article/view/98449
work_keys_str_mv AT josealexandremarzagaobarbuto hemophagocyticlymphohistiocytosisararediagnosisanevenrareropportunitytoappraiseourunderstandingoftheimmunesystem
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