Peripheral hemophagocytosis- A key to decifer hemophagocytic lymphohistiocytosis, an extremely rare case report

Hemophagocytic lymphohistiocytosis(HLH) is an uncommon and probably underdiagnosed disorder that requires quick medical attention because of its high and rapid mortality rate. The present criteria are the only ones used to diagnose HLH. For a laboratory diagnosis of HLH-2007(an upgraded version of HLH-2004), five of the eight requirements must be satisfied. We report an exceedingly rare case of a thirty-year-old Indian man who has been clinically presenting with a high-grade fever, weakness, oligouria, and speech slurring for 20 days. While screening peripheral blood smear(PBS) in order to correct complete blood counts, peripheral circulating hemophagocytic histiocytes were identified and further testing was advised as suspicion of HLH was taken into account. Our case fulfilled the WHO 2009 diagnostic criteria of HLH, hence a diagnosis of Hemophagocytic Lymphohistiocytosis was awarded. To the best of our knowledge, no HLH case has ever been recorded in India based on suspicion raised by PBS findings. Globally, only six similar cases of HLH with peripheral findings have been posted online.