Haemophagocytic Lymphohistiocytosis: A Review Article

Background: Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by the aggressive non-malignant proliferation of activated macrophages and histiocytes, which phagocytose other cells, namely red blood cells, white blood cells and platelets leading to the clinical symptoms of fever, hepato-splenomegaly, bone marrow, skin, and central nervous system (CNS) infiltration. The presumed underlying pathogenesis is a highlystimulated but ineffective immune response to antigens, mediated mainly by activated Tcells and associated macrophage activation that eventually heightens to a lifethreatening cytokine storm. Interpretation: In this case series, we present 2 cases of HLH their different presentations, clinical course and outcome. Conclusion: HLH is not an uncommon entity and it has multifaceted clinical presentation with nonspecific signs and symptoms that are often found in other clinical conditions. Therefore, there is need to know the importance of its Incidence in Indian scenario, specific management guidelines and prognosis to prevent high mortality and adverse outcomes.