Diagnosing and treating hemophagocytic lymphohistiocytosis in the tropics: Systematic review from the Indian subcontinent

Srinivas Rajagopala, Navneet Singh

Abstract


Background. Hemophagocytic lymphohistiocytosis (HLH) is a catastrophicsyndrome of unrestrained immune activation. Evaluationand management of HLH in the tropics is challenging. Objectives. Toexamine the reported etiologies and management of HLH reportedfrom the sub-continent. Methods. Systematic review of all publishedcases from the Indian sub-continent. Results. We found only 156 publishedcases of HLH from the sub-continent. HLH was reported fromthe immediate perinatal period to 46 years of age. Infection-associatedHLH (IAHS) constituted 46.8% of all cases of HLH (44% and 51%in children and adults respectively). In adults, tropical infections triggered51% of these cases of IAHS. Steroids were used in 47% of childrenand 10% of adults. Etoposide and/or cyclosporine were used in8% children and 8% of adults only. Intravenous immunoglobulin wasused in another 30% of children and 4% of the adults. HLH-relatedmortality occurred in 31.8% and 28% of children and adults respectively.Conclusions. HLH is under-reported in the sub-continentand has high mortality. Cyclosporine and etoposide are seldom administeredearly despite diagnosis of HLH. Larger cohorts with IAHStriggered by tropical infections are urgently needed to understand itsnatural history and implications of this differing prescription patternon mortality.

Keywords


Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; India; Indian sub-continent

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References


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