Hemophagocytic lymphohistiocytosis (HLH), is an uncommon hematologic disorder. It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages characterised by proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes.

Hemophagocytic lymphohistiocytosis is a rare but life threatening condition characterised by activation of macrophages which result in phagocytosis of RBCs and cytokine mediated tissue damage. This presentation aims to discuss the genetic basis, clinical features, diagnostic criteria and management options in this serious condition. The management options in HLH include Etoposide, Dexamethasone, Cyclosorine, Tacrolimus, Alemtuzumab and stem cell transplant.

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