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Hemophagocytic lymphohistiocytosis: review of etiologies and management

Authors George M

Received 27 December 2013

Accepted for publication 8 March 2014

Published 12 June 2014 Volume 2014:5 Pages 69—86

DOI https://doi.org/10.2147/JBM.S46255

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 4


Melissa R George

Department of Pathology, Penn State Milton S Hershey Medical Center, Hershey, PA, USA

Abstract: Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection. Familial forms result from genetic defects in natural killer cells and cytotoxic T-cells, typically affecting perforin and intracellular vesicles. HLH is likely under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt at curative therapy to be made. Current treatment regimens include immunosuppression, immune modulation, chemotherapy, and biological response modification, followed by hematopoietic stem-cell transplant (bone marrow transplant). A number of recent studies have contributed to the understanding of HLH pathophysiology, leading to alternate treatment options; however, much work remains to raise awareness and improve the high morbidity and mortality of these complex conditions.

Keywords: macrophage activation syndrome, hyperinflammatory response

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