儿童伤寒并发噬血细胞综合征 1 例临床分析

目的分析儿童伤寒并发噬血细胞综合征(Ty-AHS)的临床特点和转归。方法回顾分析1例Ty-AHS患儿的临床资料,并复习相关文献。结果患儿,男,4岁,持续腹泻、高热与低体温交替、表情淡漠、肝脾肿大、急性腹膜炎表现、腹腔积液。血常规示嗜酸性粒细胞为0,血红蛋白、血小板明显下降,C反应蛋白、降钙素原明显升高,血浆纤维蛋白原下降至0.8 g/L,乳酸脱氢酶升高3 835 U/L,血清铁蛋白1 884 ng/mL,血三酰甘油2.42 mmol/L,血EBV-DNA滴度2.81×10~4/m L,血培养伤寒沙门菌血清型Ⅲb;腹部彩超提示肠系膜淋巴结增大,中量腹腔积液;胸片提示肺炎;淋巴细胞分析示CD4~+/CD8~+比值降低,CD3~-CD16~+56~+细胞降低,CD19~+细胞降低;骨髓细胞学检查,骨髓增生明显活跃,粒、红、巨三系均未见明显异常,全片见较多组织细胞及噬血组织细胞。给予抗感染,地塞米松治疗2周后患儿症状消失,体征和实验室检查渐恢复正常。结论儿童Ty-AHS是一种罕见并发症,起病急,进展快,抗生素加激素治疗有效。

Clinical analysis of typhoid fever complicated with hemophagocytic syndrome in one case

Objectives To analyze the clinical features and outcome of typhoid fever complicated with hemophagocytic syndrome (Ty-AHS) in children.Methods The clinical data from one case of Ty-AHS was retrospectively analyzed, and related articles were reviewed.Results A 4-year-old boy suffered from persistent diarrhea, alternating high and low temperature, apathia, hepatosplenomegaly, manifestation of acute peritonitis, and pyoperitoneum. Routine blood examination showed that eosinophil was 0, and hemoglobin and platelet were obviously decreased; CRP and procalcitonin were obviously increased;plasma ifbrinogen was dropped to 0.8 g/L; lactate dehydrogenase was elevated to 3835 U/L; Serum ferritin was 1884 ng/mL;triglyceride was 2.42 mmol/L; EBV-DNA titer was 2.81×104 copies/mL; Blood culture showed salmonella enterica serotype IIIb. Abdominal ultrasonography showed enlargement of mesenteric lymph node and middle volume of pyoperitoneum. Chest X-ray showed pneumonia. Lymphocyte analysis showed that the ratio of CD4+/CD8+ was decreased; CD3-CD16+56+ cell and CD19+ cell were all decreased. Bone marrow cytomorphologic examination revealed that bone marrow hyperplasia was active, there were no obvious abnormalities in granulocyte, macrophage and macrophage and there were a lot of tissue cells and white blood cells. After two weeks of strengthened anti-infection and dexamethasone treatment, the symptoms in patients were disappeared, and signs and laboratory tests gradually returned to normal.Conclusions Ty-AHS is a rare complication in children with acute onset and rapid progression, and combination of antibiotics and hormone therapy is effective.