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中华重症医学电子杂志 ›› 2022, Vol. 08 ›› Issue (02) : 180 -186. doi: 10.3877/cma.j.issn.2096-1537.2022.02.015

病例报告

真性红细胞增多症并肠梗阻、抗磷脂综合征一例
李周平1, 薛晓艳1,(), 徐庆杰1, 杜扬帆1   
  1. 1. 100049 北京,北京大学航天临床医学院 航天中心医院重症医学科
  • 收稿日期:2021-07-22 出版日期:2022-07-04
  • 通信作者: 薛晓艳

One case of polycythemia vera with intestinal obstruction and antiphospholipid antibody syndrome

Zhouping Li1, Xiaoyan Xue1,(), Qingjie Xu1, Yangfan Du1   

  1. 1. Department of Critical Care Medicine, Aerospace Central Hospital, School of Aerospace Clinical Medicine, Peking University, Beijing 100049, China
  • Received:2021-07-22 Published:2022-07-04
  • Corresponding author: Xiaoyan Xue
引用本文:

李周平, 薛晓艳, 徐庆杰, 杜扬帆. 真性红细胞增多症并肠梗阻、抗磷脂综合征一例[J/OL]. 中华重症医学电子杂志, 2022, 08(02): 180-186.

Zhouping Li, Xiaoyan Xue, Qingjie Xu, Yangfan Du. One case of polycythemia vera with intestinal obstruction and antiphospholipid antibody syndrome[J/OL]. Chinese Journal of Critical Care & Intensive Care Medicine(Electronic Edition), 2022, 08(02): 180-186.

静脉血栓栓塞性疾病是住院患者常见的并发症之一,具有发生率高、病死率高和住院费用增加等特点。本文报道1例67岁既往有真性红细胞增多症(PV)病史的男性,进食不当后出现腹胀、腹痛。增强CT显示小肠梗阻(SBO)、肠穿孔、阑尾炎和腹膜炎。经禁食、放置肠梗阻减压管、药物抗感染等非手术治疗后,肠梗阻症状改善且病灶局限化。但患者随后出现以左侧锁骨下静脉、颈内静脉和上肢静脉为主的血栓。笔者考虑肠梗阻导致的脓毒症和PV可能是静脉血栓形成的主要因素,给予普通肝素抗凝、羟基脲治疗PV。实验室化验血清抗心磷脂抗体、抗β2糖蛋白1抗体、狼疮抗凝物阳性。经积极的抗凝、糖皮质激素、羟氯喹和抗感染治疗,患者病情改善。出院后随访半年,患者大部分血栓消失。抗磷脂综合征(APS)和骨髓增生性肿瘤(MPN)均与血栓形成风险增加有关,但二者同时存在的报道并不多见。早期针对血栓的病因诊断及治疗对改善病程、预后至关重要。

Venous thromboembolic disease is one of the common complications of hospitalized patients and is associated with high morbidity, high mortality and increased hospitalization costs. We reported the case of a 67-year-old male patient with a history of polycythemia vera (PV), who presented with abdominal distension, abdominal pain after improper diet. A contrast-enhanced computed tomography scan showed small bowel obstruction, perforation, appendicitis, and peritonitis. He were given non-operative treatmentincluding fasting, decompression tube placement, and anti-infection. The symptoms of ileus improved and the lesion was localized, but thrombosis was found in the subclavian vein, internal jugular vein, and upper limb vein on the left. Both of the sepsis and PV were thought to contribute to thrombosis. Heparin was used for anticoagulation and hydroxyurea for PV. Further laboratory tests revealed that serum antiphospholipid antibodies, anti-β2-glycoprotein Ⅰ antibodies and lupus anticoagulant were positive. With a combination therapy of anticoagulation, steroid, hydroxychloroquine and antibiotic, the patient's condition rapidly improved. Most venous thrombosis disappeared at half a year outpatient follow-up. Antiphospholipid syndrome (APS) and myeloproliferative neoplasms (MPN) are both associated with increased risk of thrombosis, but co-existence of these two conditions is rarely reported. Early diagnosis and treatment of thrombotic etiology are crucial to improve the course and prognosis of thrombosis.

图1 真性红细胞增多症患者腹部CT检查。图a~c:2021年5月9日增强CT示小肠置管术后,肠腔扩张,多发气液平;远段回肠肠壁增厚伴强化,肠腔内可见多发高密度灶,近端小肠扩张,多发气液平面,阑尾增粗伴强化,周围渗出改变;图d~f:2021年5月16日增强CT示腹腔多发渗出及局部包裹及含气影,增强后壁强化,腹膜轻度增厚,肠梗阻导管头端位于中腹部;回肠肠壁增厚伴强化,腹腔多发包裹性改变,局部含气,囊壁增厚并强化,阑尾增粗伴强化,周围少量渗出改变;图g~i:2021年6月2日CT示腹腔及腹壁多发包裹性低密度灶,上腹部小肠肠管扩张,可见气液平;阑尾增粗,周围少量渗出
图2 真性红细胞增多症患者不同时间段白细胞、血红蛋白、血小板变化趋势图
图3 真性红细胞增多症患者不同时间段D二聚体、活化部分凝血活酶时间变化趋势图
表1 真性红细胞增多症患者血管超声、增强CT发现静脉血栓形成情况
表2 真性红细胞增多症患者抗磷脂抗体化验结果
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