Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2019 Nov;66(11):e27929.
doi: 10.1002/pbc.27929. Epub 2019 Jul 24.

Challenges in the Diagnosis of Hemophagocytic Lymphohistiocytosis: Recommendations From the North American Consortium for Histiocytosis (NACHO)

Affiliations
Review

Challenges in the Diagnosis of Hemophagocytic Lymphohistiocytosis: Recommendations From the North American Consortium for Histiocytosis (NACHO)

Michael B Jordan et al. Pediatr Blood Cancer. .

Abstract

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.

Keywords: hematology; hemophagocytic lymphohistiocytosis; immunology.

Similar articles

See all similar articles

Cited by 1 article

MeSH terms

LinkOut - more resources

Feedback