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Pediatr Blood Cancer. 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).

Author information

1
Division of Immunobiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio.
2
Division of Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio.
3
Division of Pediatric Hematology and Oncology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
4
Division of Hematology, Children's National Medical Center, Washington, DC.
5
Center for Cancer and Blood Disorders, Phoenix Children's Hospital, University of Arizona College of Medicine, Tucson, Arizona.
6
Department of Pediatrics, UCSF Benioff Children's Hospital, University of California San Francisco, San Francisco, California.
7
Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio.
8
Division of Critical Care, Department of Pediatric Medicine, St Jude Children's Research Hospital, Memphis, Tennessee.
9
Center for Cancer and Immunology Research, Children's National Medical Center and George Washington University School of Medicine, Washington, DC.
10
Division of Cancer Predisposition, Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.
11
Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.
12
Department of Global Pediatric Medicine, St. Jude Children's Research Hospital, Memphis, Tennessee.
13
Division of Oncology, Center for Cancer and Blood Disorders, Children's National Health System, Washington, DC.

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

PMID:
31339233
DOI:
10.1002/pbc.27929

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