Coagulation Disorders in Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe condition that can lead patients to the intensive care unit. HLH diagnosis may be challenging, as it relies on sets of aspecific criteria. Several organ dysfunctions have been described during HLH, including hemostasis impairment found in more than half of the patients. The most frequently reported anomaly is a decrease in the fibrinogen level, which has been associated with higher mortality rates. Coagulation impairment study in patients with HLH represents an interesting field of research, as little is known about the mechanism leading to hypofibrinogenemia.

Keywords: Coagulation; Fibrinolysis; Hemophagocytic lymphohistiocytosis; Hemorrhage.