Hemophagocytic lymphohistiocytosis (HLH) is a rapidly progressive and life-threatening syndrome of pathologic immune activation that results in uncontrolled cytokine release and tissue destruction. HLH may be due to an underlying genetic defect or be triggered by infection, malignancy, or autoimmune disorders.
Early recognition and prompt initiation of treatment are essential for survival; without effective therapy, the majority of patients die within two months due to multi-organ failure. Diagnosis can be challenging, however, due to the rarity of the disorder, variable presentation, and non-specific clinical and laboratory findings. Treatment focuses on eliminating the underlying cause and suppressing excessive immune activation. Conventional therapies include aggressive supportive care, immunosuppressive agents (etoposide, dexamethasone, and cyclosporine), and stem cell transplantation. Several targeted therapies are currently being tested that may improve survival and avoid the myelosuppressive effects of conventional agents. Through a review of several case studies, you will gain a greater understanding of disease pathology, diagnostic workup, and treatment options for this rare and complex disorder.
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- Karyn Brundige, MSN CPNP
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