Hemophagocytic Lymphohistiocytosis: Lessons Learned from the Dark Side

Deepak Chellapandian

doi:10.1016/j.iac.2020.04.003

Hemophagocytic Lymphohistiocytosis: Lessons Learned from the Dark Side

Deepak Chellapandian

All Children's Hospital

Research output: Contribution to journal › Review article › peer-review

1 Scopus citations

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe form of immune dysregulation often presenting as unremitting fever, cytopenia, hepatosplenomegaly, coagulopathy, and elevation of typical HLH biomarkers. HLH is universally fatal, if left untreated. The HLH-2004 criteria are widely used to diagnose this condition, but there is growing concerns across different settings that its application may result in undertreatment of certain patients. There is an expanding spectrum of genetic conditions that can be complicated by HLH. This review summarizes the current concepts in HLH, the lessons learned from the past, and provide an overview of the latest diagnostic and treatment modalities.

Original language	English (US)
Pages (from-to)	485-497
Number of pages	13
Journal	Immunology and Allergy Clinics of North America
Volume	40
Issue number	3
DOIs	https://doi.org/10.1016/j.iac.2020.04.003
State	Published - Aug 2020

Keywords

Flow cytometry-based primary immunodeficiency disorder screening tests
Hematopoietic cell transplantation
Hemophagocytic syndrome
Hyperinflammation
Primary immunodeficiency
Secondary hemophagocytic lymphohistiocytosis
Targeted therapy

ASJC Scopus subject areas

Immunology and Allergy
Immunology

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10.1016/j.iac.2020.04.003

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title = "Hemophagocytic Lymphohistiocytosis: Lessons Learned from the Dark Side",

abstract = "Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe form of immune dysregulation often presenting as unremitting fever, cytopenia, hepatosplenomegaly, coagulopathy, and elevation of typical HLH biomarkers. HLH is universally fatal, if left untreated. The HLH-2004 criteria are widely used to diagnose this condition, but there is growing concerns across different settings that its application may result in undertreatment of certain patients. There is an expanding spectrum of genetic conditions that can be complicated by HLH. This review summarizes the current concepts in HLH, the lessons learned from the past, and provide an overview of the latest diagnostic and treatment modalities.",

keywords = "Flow cytometry-based primary immunodeficiency disorder screening tests, Hematopoietic cell transplantation, Hemophagocytic syndrome, Hyperinflammation, Primary immunodeficiency, Secondary hemophagocytic lymphohistiocytosis, Targeted therapy",

author = "Deepak Chellapandian",

note = "Funding Information: This work is supported by Johns Hopkins All Children{\textquoteright}s Foundation Institutional Research Grant Program to D. Chellapandian. Publisher Copyright: {\textcopyright} 2020 Elsevier Inc.",

year = "2020",

month = aug,

doi = "10.1016/j.iac.2020.04.003",

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AB - Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe form of immune dysregulation often presenting as unremitting fever, cytopenia, hepatosplenomegaly, coagulopathy, and elevation of typical HLH biomarkers. HLH is universally fatal, if left untreated. The HLH-2004 criteria are widely used to diagnose this condition, but there is growing concerns across different settings that its application may result in undertreatment of certain patients. There is an expanding spectrum of genetic conditions that can be complicated by HLH. This review summarizes the current concepts in HLH, the lessons learned from the past, and provide an overview of the latest diagnostic and treatment modalities.

KW - Flow cytometry-based primary immunodeficiency disorder screening tests

KW - Hematopoietic cell transplantation

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KW - Hyperinflammation

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KW - Targeted therapy

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Hemophagocytic Lymphohistiocytosis: Lessons Learned from the Dark Side

Abstract

Keywords

ASJC Scopus subject areas

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