Treating haemophagocytic lymphohistiocytosis

Haemophagocytic lymphohistiocytosis (HLH), a potentially\udfatal disorder, may occur as a primary genetic abnormality or\udsecondary to a number of diseases, including malignancies,\udinfections, and connective tissue disorders. The clinical course and outcome of underlying disease is often dramatically altered in the presence of secondary HLH. Although data on incidence in adults are inadequate, paediatric HLH is estimated to occur in 1–225 per 300,000 live births. 1–3 There is growing evidence that HLH is probably an underdiagnosed condition, primarily due to lack of awareness. Consequently, apart from haematologists and physicians practicing at tertiary care hospitals, this entity seldom crosses the minds of general practitioners. This leads to considerable delay in diagnosing HLH, especially in patients presenting with fever of unknown origin or cytopenias. Such patients get referred to specialists usually after a battery of infectious and inflammatory disorder workups, and at times after a course of empiric antibiotic therapy