Hemophagocytic Lymphohistiocytosis (HLH)

Hemophagocytic lymphohistiocytosis is a terminology that is being encountered frequently in the past few years. It is a pathological entity where uncontrolled inflammation results in activation of lymphocytes. Activated T lymphocytes and natural killer cells release cytokines which result in both biochemical (raised ferritin, raised triglycerides, transaminitis, hyponatremia) and pathological (pancytopenia, coagulopathy) consequences. 

HLH is the outcome of a disease rather than a disease per se. Once diagnosed, refer to tertiary care centre as mortality is very high. Over the years, management of sepsis has improved.  This is probably why we  encounter HLH more frequently . Since this is a consequence of inflammation, management would include immunosuppression to reduce inflammatory triggers. HLH should be managed in centers capable of handling immunosuppressed patients as these patients would require supportive care and prophylactic antimicrobials often akin to that needed for hematological malignancies. 

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