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Hemophagocytic lymphohistiocytosis (HLH): a review of literature

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease in children and an exceptionally rare occurrence in adults. It is categorized broadly into primary (familial) or the secondary types; the latter being associated most commonly with an underlying malignancy. HLH carries a high rate of mortality, and the treatment itself is associated with significant morbidity and risk of mortality. A high degree of suspicion for the diagnosis, early treatment and aggressive supportive care is critical for management. We present a comprehensive review of literature describing the clinical features, diagnosis, management and outcome of HLH.

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Abbreviations

HLH:

Hemophagocytic lymphohistiocytosis

F-HLH:

Familial hemophagocytic lymphohistiocytosis

HSCT:

Hematopoietic stem cell transplant

MAS:

Macrophage activation syndrome

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Correspondence to Rohtesh S. Mehta.

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Mehta, R.S., Smith, R.E. Hemophagocytic lymphohistiocytosis (HLH): a review of literature. Med Oncol 30, 740 (2013). https://doi.org/10.1007/s12032-013-0740-3

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