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J Blood Med. 2014 Jun 12;5:69-86. doi: 10.2147/JBM.S46255. eCollection 2014.

Hemophagocytic lymphohistiocytosis: review of etiologies and management.

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


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.


hyperinflammatory response; macrophage activation syndrome

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