A Case Report on Hemophagocytic Lympho Histiocytosis (HLH)

Hemophagocytic lympho histiocytosis (HLH) rare immunological disease, that related life- threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection. Familial forms result from genetic defects in natural killer cells and cytotoxic T-cells, typically affecting perforin and intra-cellular vesicles. The mechanism involves an inherited or acquired defect in the handling of antigenic factors (infectious, cancerous, or autoimmune) that leads to a severe systemic inflammatory process due to T-lymphocyte proliferation, cytokine over production and massive macrophage activation). The main symptoms ofHLH are prolonged fever, neutro-and thrombocytopenia, hepatosplenomegaly, and conspicuous laboratory values, such aslow fibrinogen and elevated levels of ferritin, triglycerides and sCD25. HLH is likely under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt atcurative therapy to be made. Current treatment regimens include immunosuppression, immune modulation, chemotherapy, and biological response modification, followed by hematopoietic stem-cell transplant. A number of recent studies have contributed to the understanding of HLH pathophysiology, leading to alternate treatment options; however, much work remains to raise awareness and improve the high morbidity and mortality of these complex conditions.