嗜血细胞综合征三例的临床资料分析并文献复习  

Analysis of clinical data of 3 cases of hemophagocytic syndrome and literature review

作　　者：李小琴[1] 薛萍[1] 郑艳梅[1] Li Xiaoqin;Xue Ping;Zheng Yanmei(Department of Pediatrics,Taiyuan Maternal and Child Health Care Hospital,Taiyuan 030001,Shanxi Province,China)

机构地区：[1]太原市妇幼保健院儿内科,030001

出　　处：《中国基层医药》2021年第9期1373-1377,共5页Chinese Journal of Primary Medicine and Pharmacy

摘　　要：目的分析3例嗜血细胞综合征(hemophagocytic syndrome,HPS)患儿的临床特点、诊断、治疗及预后,为合理治疗及预防严重并发症的发生提供诊疗线索,减少误诊误治。方法回顾性分析太原市妇幼保健院2018年3月至2020年3月收治的HPS患儿3例的临床资料,检索文献,结合病例合理归纳并解释相关结果。结果3例患儿均以发热为首发症状,均为高热(≥39℃);3例患儿最后均出现不同程度的两系或三系减少;2例患儿出现肝脾肿大,1例未发现肝脾肿大;3例患儿均有转氨酶升高,2例较严重,1例轻微;3例均有铁蛋白明显升高及纤维蛋白原降低,均无高甘油三酯血症;2例在发热过程中有皮疹出现,1例皮疹出现较晚,1例未见皮疹出现;3例患儿均有不同程度的淋巴结肿大。3例患儿中2例行骨穿检查明确,1例骨髓象见网状细胞吞噬血细胞现象,1例骨髓涂片可见利什曼原虫,2例经治疗后均治愈出院;1例因病情进展快死于多脏器功能衰竭,尸检发现肾上腺肿物。结论HPS的临床表现多样,病情凶险,病因复杂,临床预后各有差异。临床如遇到不明原因发热伴血细胞减少者,应考虑到此病的可能,需详细询问病史及生活居住史,尽早完善相关辅助检查。Objective To analyze the clinical characteristics,diagnosis,treatment and prognosis of three children with hemophagocytic syndrome(HPS)and provide diagnosis and treatment clues for reasonable treatment and prevention of serious complications and reduction of misdiagnosis and mistreatment.Methods The clinical data of three children with HPS who received treatment in Taiyuan Maternal and Child Health Care Hospital from March 2018 to March 2020 were retrospectively analyzed.Related literature was retrieved.The clinical data of the three children were summarized to analyze the outcomes.Results Fever(≥39℃)was the first symptom in all three cases.In the end,red blood cell line,white blood cell line,and platelet line were reduced to different degrees in all three cases.Hepatosplenomegaly was found in two cases.Transaminase highly increased in two cases,and slightly increased in one case.Ferritin greatly increased,fibrinogen decreased,and hypertriglyceridemia did not occur in all three cases.Two cases had skin rash during fever.Skin rash appeared late in one of them.All three cases had different degrees of lymphadenopathy.Bone puncture examination showed reticulocyte phagocytosis in bone marrow in one case and leishmania in bone marrow smear in another case.These two cases were cured and discharged.One case died of multiple organ failure due to rapid disease progression,and adrenal masses were found at autopsy.Conclusion HPS has diverse clinical manifestations,complex etiology and different clinical prognoses.HPS should be considered in case of unexplained fever with hemocytopenia.The medical history and living history should be inquired in detail and relevant auxiliary examinations should be improved as soon as possible.

关 键 词：发热 淋巴组织细胞增多症 嗜血细胞性 利什曼病 内脏 免疫 病史记录 

分 类 号：R725.5[医药卫生—儿科]