Skinner Janice, Yankey Benedicta, Shelton Brenda K
Janice Skinner is Adult Nurse Practitioner, School of Medicine, Department of Medicine Division of Hematology, Johns Hopkins Bayview Medical Center, Sydney Kimmel Cancer Center, 300 Mason Lord Dr, Baltimore MD 21224 (
AACN Adv Crit Care. 2019 Summer;30(2):151-164. doi: 10.4037/aacnacc2019463.
Hemophagocytic lymphohistiocytosis is a life-threatening condition associated with hyperinflammation and multiple organ dysfunction. It has many causes, symptoms, and outcomes. Early recognition is critical for treatment. Fever, cytopenias, coagulopathy, and hepatosplenomegaly are hallmark findings. Identifying the trigger event is crucial but challenging because of the varied presentations and infrequent provider experience. Diagnostic features include anemia, thrombocytopenia, neutropenia, elevated ferritin, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis (in bone marrow, spleen, or lymph nodes), low or absent natural killer cells, and elevated soluble interleukin 2 receptor assay. Primary treatment goals are eliminating the underlying trigger and suppressing hyperinflammation with steroids, immunoglobulins, or immunomodulators. Specific treatment includes corticosteroids, etoposide, and antithymocyte globulin followed by hematopoietic stem cell transplantation in patients with refractory or relapsing disease. Prompt immunochemical therapy is essential but often complicated by a high risk of treatment-related morbidity and disease recurrence. Despite these challenges, improvements in diagnostic technology and treatment have enhanced survival.
噬血细胞性淋巴组织细胞增生症是一种与过度炎症反应和多器官功能障碍相关的危及生命的疾病。它有多种病因、症状和转归。早期识别对治疗至关重要。发热、血细胞减少、凝血功能障碍和肝脾肿大是其标志性表现。由于临床表现多样且医生经验不足，确定触发事件至关重要但具有挑战性。诊断特征包括贫血、血小板减少、中性粒细胞减少、铁蛋白升高、高甘油三酯血症、低纤维蛋白原血症、噬血细胞现象（在骨髓、脾脏或淋巴结中）、自然杀伤细胞减少或缺乏以及可溶性白细胞介素2受体检测值升高。主要治疗目标是消除潜在触发因素并用类固醇、免疫球蛋白或免疫调节剂抑制过度炎症反应。具体治疗包括使用皮质类固醇、依托泊苷和抗胸腺细胞球蛋白，对于难治性或复发性疾病患者随后进行造血干细胞移植。及时的免疫化学治疗至关重要，但常常因治疗相关的高发病率风险和疾病复发而变得复杂。尽管存在这些挑战，但诊断技术和治疗方面的进步提高了生存率。
