Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening condition characterized by uncontrolled inflammation. The management of HLH is challenging, in part, due to the multiple etiologies of the disease, the variations in presentations, and the rapidity of the progression of the disease. Due to the severity of their disease, these patients often require significant critical care support. HLH can be due to familial (genetic) causes or can be secondary to triggers such as infections, autoimmune disorders, and malignancy. Underlying conditions such as sepsis or malignancy could pose as major confounders while applying universal diagnostic criteria and therefore could lead to delay in diagnosis. This chapter focuses on the characteristics of the disease, pathophysiology, diagnostic criteria, treatment, and intensive care management of pediatric patients with HLH.
CITATION STYLE
Hines, M., Bhatt, N., & Talano, J. A. M. (2019). Diagnosis, Treatment, and Management of Hemophagocytic Lymphohistiocytosis in the Critical Care Unit. In Critical Care of the Pediatric Immunocompromised Hematology/Oncology Patient: An Evidence-Based Guide (pp. 159–182). Springer International Publishing. https://doi.org/10.1007/978-3-030-01322-6_9
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