Hemophagocytic lymphohistiocytosis as an etiology of bone marrow failure.

bone marrow failure (BMF) cytopenia familial hemophagocytic lymphohistiocytosis (FHL) hemophagocytic lymphohistiocytosis hypercytokinaemia macrophage activation syndrome (MAS)

Journal

Frontiers in oncology
ISSN: 2234-943X
Titre abrégé: Front Oncol
Pays: Switzerland
ID NLM: 101568867

Informations de publication

Date de publication:
2022
Historique:
received: 10 08 2022
accepted: 27 09 2022
entrez: 17 11 2022
pubmed: 18 11 2022
medline: 18 11 2022
Statut: epublish

Résumé

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of multiorgan system dysfunction that is caused by hypercytokinemia and persistent activation of cytotoxic T lymphocytes and macrophages. A nearly ubiquitous finding and a diagnostic criterion of HLH is the presence of cytopenias in ≥ 2 cell lines. The mechanism of cytopenias in HLH is multifactorial but appears to be predominantly driven by suppression of hematopoiesis by pro-inflammatory cytokines and, to some extent, by consumptive hemophagocytosis. Recognition of cytopenias as a manifestation of HLH is an important consideration for patients with bone marrow failure of unclear etiology.

Identifiants

pubmed: 36387094
doi: 10.3389/fonc.2022.1016318
pmc: PMC9647152
doi:

Types de publication

Journal Article Review

Langues

eng

Pagination

1016318

Subventions

Organisme : NHLBI NIH HHS
ID : T32 HL007574
Pays : United States

Informations de copyright

Copyright © 2022 Paolino, Berliner and Degar.

Déclaration de conflit d'intérêts

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Auteurs

Jonathan Paolino (J)

Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, United States.

Nancy Berliner (N)

Division of Hematology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States.

Barbara Degar (B)

Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, United States.

Classifications MeSH