Abstract
Hemophagocytic Lymphohistiocytosis (HLH) is a rare and life threatening syndrome caused by excessive and dis-regulated immune activation. It can present as a primary sporadic disorder or be secondary to a trigger disrupting the immune homeostasis, such as auto-immune disorders, malignancies and mainly infections. It is usually of low suspicion index, and has variable presentations lacking specific pathognomonic clinical or laboratory signs. HLH is a medical emergency, and is associated with poor prognosis in most of the cases. An early diagnosis and initiation of appropriate treatment may change the outcome. Here I present a review of the literature concerning HLH, and one related pathologically similar disorder, the Macrophage Activation Syndrome (MAS), emphasizing on the clinical presentation, associated etiologies, diagnosis, treatment and prognosis, thus making the clinicians more aware of this fatal syndrome in order to decrease the related mortality.
Citation
EI Hachem G. Hemophagocytic Lymphohistiocytosis, A Syndrome of Excessive Immune Activation: Review of the Literature. SM J Hematol Oncol. 2017; 2(1): 1006s.