发热，肝脾肿大，下肢无力
Q4 Medicine
引用次数: 0
摘要
Patient female, 22 years old. Due to recurrent fever for over a year and weakness in both lower limbs for over 20 days, he was admitted to our hospital on October 26, 2016. The patient experienced recurrent fever without any obvious cause more than a year ago (May 2015), with a maximum body temperature of 40 ° C. Laboratory examination in an external hospital showed a blood routine white blood cell count of 1.04 × 10 9/L [(3.50~9.50) × 10 9/L], reticulocyte ratio 0.0145 (0.80-2.00), hemoglobin 92 g/L (110-150 g/L), platelet count 72 × 10 9/L [(100~350) × 10 9/L]; Detection of EB virus DNA in peripheral blood 416 × 10 3 copies/ml; Abdominal ultrasound shows that the right oblique diameter of the liver is 16.10 cm, the inner diameter of the portal vein is 1.30 cm, the thickness of the spleen at the splenic hilum is 7.40 cm, the inner diameter of the splenic vein is 1 cm, the lateral position is 16 cm below the splenic rib, and the supine position is 5 cm above the midline of the spleen, indicating liver enlargement, diffuse changes in the liver parenchyma, enlarged spleen, and mild dilation of the portal vein system; Chest CT shows multiple nodular shadows in both lungs, indicating inflammatory changes; Bone marrow cytology examination showed three lines of proliferative bone marrow, with an increase in the proportion of red blood cells; Bone marrow biopsy indicates that bone marrow hyperplasia is generally normal, while tertiary hyperplasia with mild abnormalities in megakaryocyte morphology; JAK2 V617F quantification 0%, CALR Exon9 and MPL Exon10 mutations negative; Flow cytometry showed an increase in the proportion of granulocytes, nuclear shift to the left, and downregulation of CD13 expression; Immunohistochemical staining showed a count of 1391 CD41+megakaryocytes, including 1238 normal megakaryocytes. Neutrophil alkaline phosphatase (NAP) and glycogen periodate Schiff (PAS) staining were negative, and the positive rate of sideroblasts was 2% (27%~94%). Clinically, EB virus infection was considered, and anti infection, liver protection, and intravenous immunoglobulin (specific plan unknown) treatment were given. After 2 months, the fever symptoms gradually relieved. Afterwards, regular blood routine examinations showed a decrease in whole blood cell count; Abdominal ultrasound shows that the liver and spleen are enlarged, and the portal vein system continues to expand. More than 20 days ago, the patient had no obvious cause of weakness in both lower limbs, and when walking, both lower limbs were heavy and stiff, with symptoms gradually worsening; Before the 14th day, it is necessary to walk with assistance and not squat on your own, as there may be occasional falls; Symptoms slightly relieved 7 days ago, with no dizziness, headache, nausea, vomiting, blurred vision, blurred speech, coughing when drinking water, numbness in limbs, or bowel and bowel movements during the course of the disease. The MRI examination of the thoracic spine in the external hospital (October 19, 2016) showed diffuse patchy abnormal signal shadows in the thoracic spinal cord, suggesting the possibility of inflammatory demyelinating lesions. In order to seek further diagnosis and treatment, we have sought medical attention at our hospital, where the outpatient department is admitted as a "spinal cord disease". Since the onset of the disease, the patient's mental state, sleep, and diet have been tolerable, their bowel movements have been normal, and their weight has not significantly decreased. They deny immune colors such as dry eyes, dry mouth, hair loss, rash, joint swelling and pain, and Raynaud's phenomenon. There is no special history, personal history, or family history. After admission, the patient underwent physical examination with a body temperature of 37.2 ° C, pulse rate of 80 beats/min, respiration rate of 23 beats/min, and blood pressure of 105/64 mm Hg (1 mm Hg=0.133 kPa). One small lymph node is palpable under the right jaw, the size of a soybean, with good mobility, tough texture, and no tenderness. The abdomen is soft, without tenderness, the liver is not palpable under the ribs, the spleen is large, and about 8 cm below the ribs. Neurological examination: clear consciousness, fluent language, normal visual acuity and field of vision, bilateral pupils of equal size and circle, with a diameter of approximately 4 mm, sensitive to light reflex, and no abnormalities in the remaining cranial nerves; Both upper limbs have muscle strength of level 5 and normal muscle tone, while both lower limbs have muscle strength of level 5 and increased muscle tone; Acupuncture sensation and tuning fork vibration sensation are normal; Bilateral finger nose test, rapid rotation movement, and stable heel knee tibia test; Active limb tendon reflex; Positive Hoffmann sign on the right and negative on the left, positive Rossolimo sign on both sides, positive Babinski sign and Chaddock sign on both sides, positive ankle clonus on both sides, and negative Romberg sign; Having a spasmodic gait; Negative meningeal stimulation sign. Neuropsychological tests: The Simplified Intelligence State Examination Scale (MMSE) scores 24 points, and the Montreal Cognitive Assessment Scale (MoCA) scores 23 points. Auxiliary laboratory examination: Blood routine white blood cell count 0.80 × 10 9/L, neutrophil count 0.49 × 10 9/L [(2.00~7.50) × 10 9/L], reticulocyte count 99.70 × 10 9/L [(24-84) × 10 9/L], reticulocyte ratio 0.0241, hemoglobin 114 g/L, platelet count 45 × 10 9/L, erythrocyte sedimentation rate (ESR) 2 mm/h (0-20 mm/h); Blood smear: Red blood cells vary slightly in size, white blood cells have generally normal morphology, and platelets are rare. Blood chemistry total bilirubin 26.50 μ Mol/L (5.10~22.20 μ Mol/L), direct bilirubin 10.40 μ Mol/L fever, liver, spleen, and lower limb weakness本文章由计算机程序翻译，如有差异，请以英文原文为准。
Fever hepatosplenomegaly weakness of lower limbs
患者 女性,22 岁。主因反复发热 1 年余,双下肢无力 20 余天,于 2016 年 10 月 26 日入我院。患者于 1 年余前 (2015 年 5 月)无明显诱因出现反复发热,体温最高达 40 °C, 外院实验室检查:血常规白细胞计数为 1.04 × 10 9/L[(3.50 ~ 9.50)× 10 9/L],网织红细胞比例 0.0145(0.80 ~ 2.00),血红蛋 白 92 g/L(110 ~ 150 g/L),血小板计数为 72 × 10 9/L[(100 ~ 350)× 10 9/L];外周血 EB 病毒 DNA 测定 416 × 10 3 拷贝/ml; 腹部超声显示,肝右斜径 16.10 cm,门静脉内径 1.30 cm,脾门 处脾厚 7.40 cm,脾静脉内径 1 cm,侧卧位脾肋下 16 cm、平卧 位脾过中线 5 cm,提示肝脏肿大,肝实质弥漫性改变,巨脾, 门静脉系统轻度扩张;胸部 CT 显示双肺多发性结节影,提示 炎症性改变;骨髓细胞学检查可见三系增生骨髓象,红系比 例增加;骨髓活检提示骨髓增生大致正常,三系增生伴巨核 细胞形态轻度异常;JAK2 V617F 定量 0%,CALR Exon9 和 MPL Exon10 突变阴性;流式细胞术显示,粒系比例增加,出 现核左移,CD13 表达下调;免疫组织化学染色,CD41+巨核细 胞计数 1391 个,其中正常巨核细胞计数 1238 个,中性粒细 胞碱性磷酸酶(NAP)和糖原高碘酸⁃雪夫(PAS)染色阴性,铁 粒幼细胞阳性率为 2%(27% ~ 94%)。临床考虑 EB 病毒感 染,遂予以抗感染、保护肝脏和静脉注射免疫球蛋白(具体方 案不详)治疗,2 个月后发热症状逐渐缓解。此后定期复查 血常规显示全血细胞计数减少;腹部超声显示,肝脾大,门静 脉系统持续扩张。患者 20 余天前无明显诱因出现双下肢无 力,行走时双下肢沉重、僵硬,症状进行性加重;14 d 前需搀 扶行走,不能自行蹲起,偶有跌倒;7 d 前症状略减轻,病程中 无头晕、头痛,无恶心、呕吐,无视物模糊、视物成双,无言语 不清、饮水呛咳,无肢体麻木,无大小便障碍。外院胸椎 MRI 检查(2016 年 10 月 19 日)显示,胸髓内弥漫性斑片状异常信 号影,考虑炎性脱髓鞘病变可能。为求进一步诊断与治疗, 至我院就诊,门诊以“脊髓病变”收入院。患者自发病以来, 精神、睡眠、饮食尚可,大小便正常,体重无明显减轻,否认眼 干、口干、脱发、皮疹、关节肿痛和雷诺现象等免疫色彩。 既往史、个人史及家族史均无特殊。 入院后体格检查 患者体温 37.2 °C,脉搏 80 次/min,呼 吸 23 次/min,血压 105/64 mm Hg(1 mm Hg = 0.133 kPa)。右 侧颌下触及 1 个小淋巴结,黄豆大小,活动度可,质地坚韧, 无压痛。腹部柔软,无压痛,肝脏肋下未触及,脾大,肋下约 8 cm。神经系统检查:神志清楚,语言流利,视力、视野粗测 正常,双侧瞳孔等大、等圆,直径约 4 mm,对光反射灵敏,余 脑神经未见异常;双上肢肌力 5 级、肌张力正常,双下肢肌力 5-级、肌张力增高;针刺觉、音叉震动觉正常;双侧指鼻试验、 快复轮替动作和跟⁃膝⁃胫试验稳准;四肢腱反射活跃;右侧 Hoffmann 征阳性、左侧阴性,双侧 Rossolimo 征阳性,双侧 Babinski 征 阳 性 、Chaddock 征 阳 性 ,双 侧 踝 阵 挛 阳 性 , Romberg 征阴性;呈痉挛步态;脑膜刺激征阴性。神经心理 学测验:简易智能状态检查量表(MMSE)评分 24 分,蒙特利 尔认知评价量表(MoCA)评分 23 分。 辅助检查 实验室检查:血常规白细胞计数 0.80×10 9/L、 中性粒细胞计数 0.49 × 10 9/L[(2.00 ~ 7.50)× 10 9/L],网织红 细胞计数 99.70 × 10 9/L[(24 ~ 84)× 10 9/L]、网织红细胞比例 0.0241,血红蛋白 114 g/L,血小板计数 45 × 10 9/L,红细胞沉降 率(ESR)2 mm/h(0 ~ 20 mm/h);血涂片:红细胞大小轻度不 等,白细胞形态大致正常,血小板少见。血液化学总胆红素 26.50 μmol/L(5.10 ~ 22.20 μmol/L),直接胆红素 10.40 μmol/L 发热 肝脾大 双下肢无力
求助全文
通过发布文献求助，成功后即可免费获取论文全文。
去求助
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
