Hemophagocyticlymphohistiocytosis( HLH) is an uncommon and likely underdiagnosed disease ,potentially life threatening, hyperinflammatory syndrome involving a final common pathway of hypercytokinemia resulting in end organ damage. Although an early diagnosis is crucial to decrease the mortality, the definitive diagnosis is often challenging in spite of the well known diagnostic criteria put forth by HLH society because of the lack of specificity of currently accepted diagnostic criteria. We describe 13 patients with secondary HLH who satisfied HLH-2009 diagnostic criteria. Definitive evidence of hemophagocytosis on initial bone marrow aspiration was present in all the cases. The underlying etiologies were EBV,bacteria ( known c/o infective endocarditis ), autoimmune disorder, malaria, SLE with HIV and associated disseminated Histoplasmosis, JIA and Lymphoma. Cause could not be ascertained in four patients. Out of thirteen patients four patients died with multiorgan failure and disseminated intravascular coagulation (DIC). Due to lack of specificity of diagnostic criteria, diagnosing and differentiating HLH from its closest mimickers like sepsis/septic shock may be quite challenging in critically ill patients. Therefore increasing awareness among physicians is essential for early diagnosis and effective therapy to reduce the mortality
