噬血细胞综合征13例临床分析

目的：了解噬血细胞综合征(HS)患者的临床特点及预后。方法：分析13例HS患者临床症状、体征、实验室资料及转归。结果：13例患者均有不同程度的肝损害，如乳酸脱氢酶及胆红素增高；凝血酶原时间延长，低纤维蛋白原血症以及顽固性低钠血症。检测5例患者的甘油三酯及铁蛋白，其浓度明显增高。使用HS患者外周血白细胞富集涂片作瑞—姬氏染色及免疫细胞化学方法发现成熟单核样组织细胞形态和吞噬血小板现象。本组感染相关HS患者死亡一例(1／6，16.6％)；非感染相关HS死亡4例(4／5，80％)。结论：HS尤非感染相关HS预后凶险，顽固性低钠血症可能是HS较早期病变特点。多次动态观察外周血及骨随中噬血细胞数量变化以及行外周血白细胞富集涂片检查有利于HS的诊断。

Clinical analysis of 13 patients with hemophagocytic syndrome]

OBJECTIVE: To investigate the clinical features and prognosis of hemophagocytic syndrome (HS). METHODS: The clinical symptoms, signs, and laboratory-test data in 13 patients with HS were analysed. RESULTS: Increase in lactate dehydrogenase (LDH) and hyponatremia was found in all of the patients. Prolonged prothrombin time, hypofibrinogenemia, hyertriglyceridemia, and hyperferricemia also existed in some cases. The mature hemophagocytic histocyte and hemophagocytic phenomenon were observed with Whrigt-Geimsa and immunocytochemical staining. One (16.6%, 1/6) patient with infectious associated HS (IAHS) and 4 (80%, 4/5) with non-IAHS died of infection and primary disease. CONCLUSION: HS especially non-IAHS is an extremely dangerous state with high mortality. Obstinate hyponatremia may be a characteristic of HS in the early stage. It's important to supervise the change of hemophagocyte in peripheral blood and bone marrow of HS. Immunocytochemical studies on smear of enriched peripheral white blood cells are helpful to identify the primary pathogenesis of the benign or malignant diseases.