Dengue Hemophagocytic Syndrome-Review Article

Dengue fever is caused by infection with any of four mosquito-transmitted dengue viruses (DENV-14) and is characterized by fever, headache, myalgia, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a potentially lethal hyperinflammatory syndrome. HLH is characterized by a constant activation of CD8+ T lymphocytes and natural killer (NK) cells to enact phagocytosis of hematopoietic cells and resulting in organ damage, affecting mainly bone marrow, liver, and nervous system. Inappropriate stimulation of macrophages in bone marrow and subsequent phagocytosis of blood cells with the production of high amounts of proinflammatory cytokines are the pathologic trademarks of HLH. Quick diagnosis and treatment are essential to prevent a fatal from various triggers. Dengue associated HLH must be suspected in the presence of persistent fever beyond the day of seven, hyperferritinemia, worsening cytopenias, shock and MOD beyond plasma leakage phase. BM biopsy should be performed to demonstrate hemophagocytosis. Prompt recognition and early institution of appropriate therapy may result in a good outcome, particularly in infection-associated HLH. The aim of the review is to highlight the diagnostic criteria, pathophysiology, and treatment of the HLH due to dengue fever