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TWO FACED HLH: COMPARATIVE REVIEW OF TWO CASES OF ADULT HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

&NA; Haemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease of children and adults. Cytokine dysfunction, uncontrolled accumulation of activated T-cells and histiocytes, and the inability to turn off the immune response lead to the clinical manifestations of extreme inflammation and end-organ damage. HLH is notoriously under-reported because of its ability to mimic many other common diseases. With improvements in molecular genetics and a better understanding of the pathogenesis of the disease, a set of diagnostic criteria can aid the clinician in recognising the disease early and instituting prompt management to prevent significant morbidity and mortality. Aim: To compare two cases of HLH presenting within 6 months at Wollongong Hospital in 2012, to highlight the differences between primary and secondary HLH and the complications which may arise from each. Method: Case histories, laboratory results and personal communication with diagnostic institutions were used to summarise the cases. A review of the current literature was performed to supplement the discussion. Results: Cases of primary and secondary HLH were compared in terms of clinical features and pathogenesis, as well as triggers and complications. Discussion: A review of the literature was performed to identify trends in current diagnostics including the advent of sophisticated genetic testing, to provide a schema for recommended diagnostic testing and to discuss therapeutic principles and emerging concepts.