Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO).
Adult
Age of Onset
Child
Clinical Trials as Topic
/ standards
Diagnosis, Differential
Disease Management
Drug Eruptions
/ etiology
Fetal Diseases
/ diagnosis
Hematopoietic Stem Cell Transplantation
Humans
Immunocompromised Host
Infant
Infant, Newborn
Liver Failure
/ etiology
Lymphohistiocytosis, Hemophagocytic
/ diagnosis
Macrophage Activation
Metabolism, Inborn Errors
/ etiology
Neoplasms
/ complications
Phenotype
Sepsis
/ etiology
hematology
hemophagocytic lymphohistiocytosis
immunology
Journal
Pediatric blood & cancer
ISSN: 1545-5017
Titre abrégé: Pediatr Blood Cancer
Pays: United States
ID NLM: 101186624
Informations de publication
Date de publication:
11 2019
11 2019
Historique:
received:
09
04
2019
revised:
10
06
2019
accepted:
28
06
2019
pubmed:
25
7
2019
medline:
26
2
2020
entrez:
25
7
2019
Statut:
ppublish
Résumé
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, often associated with genetic defects of lymphocyte cytotoxicity. Though a distinctive constellation of features has been described for HLH, diagnosis remains challenging as patients have diverse presentations associated with a variety of triggers. We propose two concepts to clarify how HLH is diagnosed and treated: within the broader syndrome of HLH, "HLH disease" should be distinguished from "HLH disease mimics" and HLH subtypes should be categorized by specific etiologic associations, not the ambiguous dichotomy of "primary" and "secondary." We provide expert-based advice regarding the diagnosis and initiation of treatment for patients with HLH, rooted in improved understanding of its pathophysiology.
Identifiants
pubmed: 31339233
doi: 10.1002/pbc.27929
pmc: PMC7340087
mid: NIHMS1600652
doi:
Types de publication
Journal Article
Practice Guideline
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
e27929Subventions
Organisme : NCI NIH HHS
ID : K12 CA090433
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA154947
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA233719
Pays : United States
Informations de copyright
© 2019 Wiley Periodicals, Inc.
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