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Hemophagocytic lymphohistiocytosis secondary to infections: a tertiary care experience

Swaroopa Deme, Bhaskar Kakarla, Naval Chandra, Subbalaxmi M. V. S., Shetty Mallikharjuna, Satyanarayana Raju Yadati.

Abstract
Background: Hemophagocytic lymphohistiocytosis is an uncommon complication of various conditions. It is characterized by immune dysregulation and massive cytokine release causing multiorgan dysfunction. It is classified as primary and secondary to various etiologies like infections, malignancies and autoimmune disease. As it has high mortality, clinician awareness is important for early diagnosis and improved outcome. Aim of the study was to study the etiologies, clinical manifestations, complications and laboratory features in patients diagnosed with infection associated hemophagocytic syndrome (IAHS).
Methods: We have done retrospective analysis of all cases diagnosed to have Infection Associated Hemophagocytic Syndrome (IAHS) between March 2012 to November 2015 in a 1000 bedded tertiary care hospital in south India.
Results: Total five cases detected. Most of the cases are related to tropical infections (80%). All of them presented with fever, cytopenias and organomegaly. Ferritin and Triglycerides were elevated in all patients. Bone marrow hemophagocytosis was observed in 80% of cases. Diagnostic protocol of HLH 2004 was followed. Only 20% survival observed.
Conclusions: IAHS is a rare fulminant complication associated with diagnostic and therapeutic challenges because of overlapping clinical features with sepsis. Increased physician awareness, early diagnosis and therapeutic interventions may improve survival.

Key words: Hemophagocytic lymphohistiocytosis, Infections, Tertiary care


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