Medical ICU, Saint Louis University Hospital, Assistance Publique des Hôpitaux de Paris, 1 Avenue Claude Vellefaux, 75010 Paris, France.
Medical ICU, Saint Louis University Hospital, Assistance Publique des Hôpitaux de Paris, 1 Avenue Claude Vellefaux, 75010 Paris, France.
Crit Care Clin. 2020 Apr;36(2):415-426. doi: 10.1016/j.ccc.2019.12.004. Epub 2020 Feb 3.
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.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见且严重的疾病，可导致患者入住重症监护病房。HLH 的诊断具有一定挑战性，因为它依赖于一系列非特异性标准。HLH 期间会出现多种器官功能障碍，超过一半的患者存在止血功能障碍。最常报道的异常是纤维蛋白原水平降低，与更高的死亡率相关。HLH 患者的凝血功能障碍研究是一个有趣的研究领域，因为人们对导致低纤维蛋白原血症的机制知之甚少。
