内脏利什曼病相关噬血细胞综合征1例

A case of visceral leishmaniasis related hemophagocytic

患者,女,60岁,农民,甘肃陇南人。因发热、乏力、纳差于2023年8月25日就诊于当地县医院,完善相关检查后考虑血液系统疾病,予以输血、抗感染等对症治疗后仍有反复发热。2023年9月7日就诊于甘肃省人民医院,入院查体:脾脏肋缘下2 cm,质韧,无触痛。血常规示:白细胞计数1.5×10^(9)/L(↓),红细胞计数3.8×10^(12)/L(↑),血红蛋白68 g/L(↓),血小板计数4.3×10^(10)/L(↓);铁蛋白>2000µg/L(↑),甘油三酯2.4 mmol/L(↑),纤维蛋白原1.4 g/L(↓);可溶性CD25为1202 U/ml(↑)。骨髓涂片细胞学分析结果可见吞噬了活体血细胞的特殊巨噬细胞;骨髓涂片镜检结果显示,部分巨噬细胞胞浆内及周围可见杜氏利什曼原虫无鞭毛体。流行病学史调查结果显示,患者长期居住于我国内脏利什曼病主要流行区的甘肃陇南礼县某山区,诊断为内脏利什曼病相关噬血细胞综合征。予以葡萄糖酸锑钠(6 ml/d)及地塞米松(15 mg/d)静脉滴注1周,患者再无发热。9月18日出院前复查,血常规示:白细胞计数4.5×10^(9)/L,红细胞计数4.3×10^(12)/L,血红蛋白80 g/L,血小板计数1.1×10^(11)/L,纤维蛋白原1.51 g/L,较入院时均升高。5个月后复诊,化验血常规示白细胞、血红蛋白、纤维蛋白原均恢复正常;血小板有所升高,但仍低于正常范围。腹部超声:肝脏、脾脏大小正常。

A 60‑year‑old female patient from Longnan of Gansu Province,was admitted to a local county hos‑pital on August 25,2023,due to fever,fatigue and poor appetite.After completing relevant examinations,she was considered to have hematological diseases.After symptomatic treatments such as blood transfusion and anti‑infection,the patient still had recurrent fever.On September 7,2023,the patient was treated in the People’s Hospital of Gansu Province.Physical examination on admission showed that the spleen was 2 cm below the costal edge,tough and without tenderness.Blood routine showed that white blood cell count was 1.5×10^(9)/L(↓),red blood cell count was 3.8×10^(12)/L(↑),hemoglobin was 68 g/L(↓),platelet count was 4.3×10^(10)/L(↓),Ferritin was over 2000µg/L(↑),triglyceride was 2.4 mmol/L(↑),fibrinogen was 1.4 g/L(↓)and sluble CD25 was 1202 U/ml(↑).The results of bone marrow cytological analysis showed that the special macrophages phagocytized living blood cells.Bone marrow smear microscopy showed that some of the macrophages had Leishmania donovani amastigotes in and around the cyto‑plasm.Combined with the epidemiological history,the patient had long lived in a mountainous area in Longnan of Gansu Province,which is the main endemic area of visceral leishmaniasis in China.The patient was diagnosed with visceral leishmaniasis related hemophagocytic syndrome.After treatment with gluconate(6 ml/d)and dexamethasone(15 ml/d)for one week,the patient had no fever.Reexamination on September 18 showed that white blood cell count was 4.5×10^(9)/L,red blood cell count was 4.3×10^(12)/L,hemoglobin was 80 g/L,platelet count was 1.1×10^(11)/L and fi‑brinogen was 1.51 g/L,all of which were higher than those at admission.Five months later,the blood test routine showed that the white blood cells,hemoglobin and fibrinogen returned to normal.Platelets were elevated,but still be‑low the normal range.Abdominal ultrasound showed that the size of the liver and spleen were normal.