Case Series of Hemophagocytic Lymphohistiocytosis from a Tertiary Care Centre: An Underdiagnosed Entity

Abstract

Objective: Hemophagocytic Lymphohistiocytosis (HLH) is an uncommon, life-threatening hyperinflammatory syndrome, caused by severe hypercytokinemia, due to an overstimulated but ineffective immune process. The presenting features of HLH are non-specific, mimicking many other diseases, and hence its early recognition still remains a challenge. It requires a high index of suspicion and detailed analysis of clinical and laboratory findings to arrive at a conclusive diagnosis. The objective of this study is to present detailed clinical and laboratory features of a series of HLH cases. Material and Method: A retrospective study was conducted wherein all the bone marrow aspirate smears diagnosed as HLH between January 2015 to June 2018 were reviewed. Detailed correlation of clinical and laboratory criteria was done with the bone marrow findings. Results: A total of twelve cases were diagnosed as HLH from January 2015 to June 2018. Ten patients fulfilled 5 out of 8 clinical and lab diagnostic criteria of HLH (2004). After correlating clinical and laboratory criteria along with bone marrow findings the diagnosis of HLH was suggested. Conclusion: We present a series of twelve cases of Hemophagocytic Lymphohistiocytosis from a tertiary care hospital in New Delhi which will add not only to the understanding of this rare life threatening disease but also to the early diagnosis and intervention.

Keywords

• Hemophagocytosis
• Lymphohistiocytosis
• Hyperferritinemia
• Pancytopenia