Hemophagocytic Syndrome: Experience in a tertiary care centre

Abstract

Introduction: Hemophagocytic syndrome also known as Hemophagocytic Lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory condition. It can be primary or a secondary disorder associated with a variety of underlying conditions. These disorders feature severe cytopenias due to uncontrolled hemophagocytosis and clinical symptoms result from disordered immune regulation and cytokine storm. Materials and Methods: The present study was done at the tertiary care centre attached to University Medical College in western India. Forty five patients with cytopenias who were suspected to have secondary HLH were included in this study. Clinical details and routine investigations, tests necessary to diagnose HLH i.e. Ferritin, Triglyceride, LDH, Fibrinogen levels were done. Bone marrow aspirate in these patients showed evidence of hemophagocytosis. Statistical analysis was done using the appropriate software and statistical tests. Results: Infection was the commonest cause associated with secondary HLH in our study. 95.5% patients had anaemia while 42.2% patients had fever along with other clinical manifestations. Elevated Serum Ferritin levels were found in 55.5% patients. 82.1% and 63.0% patients had elevated Lactate Dehydrogenase and elevated triglycerides levels respectively while only 35.6% patients had low fibrinogen levels. Higher levels of ferritin were associated with severe cytopenias and with poor outcome of the patients. PT and APTT were also deranged in a significant proportion of patients. We have also observed raised APTT values correlating with abnormal ferritin levels and cytopenias in our study. This could be because most of these cases were associated with sepsis. We found significant association of elevated serum Ferritin, Triglyceride and LDH levels with HLH. Conclusion: Hemophagocytic syndromes should be considered in unexplained cytopenias. A bone marrow aspiration or organ biopsy revealing hemophagocytosis along with Serum Ferritin, Triglycer