获得性依赖维生素K凝血因子缺乏症62例临床特征及误诊分析

Analysis of the cinical features and misdiagnosis in 62 patients with acquired deficiency of vitamin K- dependent coagulation factors

目的探讨非婴幼儿获得性依赖维生素K凝血因子缺乏症的临床特征及误诊原因。方法回顾性分析我院诊治的62例获得性依赖维生素K凝血因子缺乏症患者的病因、临床表现、治疗、随访结果及误诊原因。结果62例患者中51例患者病因不明，11例为杀鼠药中毒。所有患者均同时或先后出现皮肤、黏膜、皮下及关节、肌肉、内脏出血，首发最常见出血症状为血尿；35例（56．5％）失血患者血红蛋白（Hb）〈100g／L，最低仅为32g/L；治疗后患者活化部分凝血活酶时间（APIT）、凝血酶原时间（PT）、国际标准化比值（INR）与治疗前比较差异均有统计学意义[（145．46±43．22）s、（36．62±7．85）s，（107．33±47．38）S、（13．64±4．86）S，（7．63±1．87）、（2．14±0．68），t值分别为19．51、15．49、13．62，P均〈0．01]，血小板计数、凝血酶时间（TT）、纤维蛋白原（№）均正常，患者自出血症状至明确诊断的中位时间为8（2～192）d，其中16例患者出血1—6个月才确诊，首诊误诊率高达61．3％（38／62），误诊为泌尿系统疾病占60．5％（23／38），其次为血友病占21．1％（8／38）。所有患者接受维生素K治疗，总疗程为1～22个月，少数严重出血患者同时使用凝血酶原复合物和（或）血浆输注。中位随访时间为8（1～56）个月，无一例患者复发。结论非婴幼儿获得性依赖维生素K凝血因子缺乏症多数原因不明。首发出血最常见的症状为血尿，本病易误诊，最常见误诊为泌尿系统疾病，早期的凝血功能检测可减少误诊。维生素K序贯治疗可有效防止复发，患者预后良好。

Objective To explore the clinical features and causes of misdiagnosis of the patients with acquired deficiency of vitamin K-dependent coagulation factors（ADVKDCF）. Methods Retrospective analysis was performed with the data from 62 patients with ADVKDCF for etiological factors, clinical manifestations, laboratory examinations, diagnosis and treatments. Results Among the 62 patients, 51 patients were with unknown causes （subgroup A） and 11 were with clear histories of anticoagulant rodenticide poisoning （subgroup B）. The presentations of hemorrhage of the patients varied with hematuria as the most common first symptom, followed by skin, mucosa, muscle, internal organs bleeding （28/62）. The most common hemorrhage symptom is hcmaturia. 35 of the 62 patients had hemoglobin（Hb） levels less than 100 g/L due to blood loss（ the lowest level was 32 g/L）. Thirty-eight patients were misdiagnosed at the first visit and the median time from hemorrhage manifestation to definite diagnosis was 8 clays （range,2 to 192 days）. ADVKDCF was mostly misdiagnosed as the urinary system diseases （23/38）, followed by hemophilia （8/38）＇. Laboratory examinations showed normal platelet count ,thrombin time （TI＇） and normal fibrinogen（Fg） concentration ,but prolonged plasma prothrombin time （PT）, activated partial prothrombin time （AP3~F） and international normalized ration （INR）. All of patients received high dose vitamin K （intravenous vitamin Kl with a initial dose of 20 to 240 mg/d and then oral vitamin K4 maintenance） . The bleeding symptoms disappeared 1 day after treatment and the Hb levels increased dramatically. There were significant differences in PT, APTT and INR of the patients before and after treatment（P 〈0. 01 ）. Followed by a median follow - up of 8 months , no patient had severe adverse effects or recurrence. Conclusion The hemorrhage presentations of the patients with ADYKDCF are various. The most common hemorrhage symptom is hematuria. The misdiagnosis rate of ADVKDCF is high with urinary systems disorders as the most common misdiaguosis. Sequential treatment with vitamin K is an effective and safe method to prevent recurrence. Early detection of coagulation function is helpful to reduce misdiaguosis possibility.