凝血因子 VII 和 IX 共同缺乏症:病例报告。

IF 1.2 4区 医学 Q4 HEMATOLOGY Blood Coagulation & Fibrinolysis Pub Date : 2024-09-01 Epub Date: 2024-07-16 DOI:10.1097/MBC.0000000000001315
Jhon Alexander Avila Rueda, Cesar de la Hoz, Edgar Fabián Manrique-Hernández
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引用次数: 0

摘要

本文介绍了一种不寻常临床情况的诊断和治疗方法。23 岁的女性患者因血尿和月经过多接受评估。她报告说,由于童年时曾有过出血症状,因此没有定期接受血液科随访。她接受 VII 因子治疗已有 2 年,但否认因出血而住院。实验室报告显示,血红蛋白:5.2 g/dl;血小板:234 000/mm3;PT:100 s;PTT:112 s,纤维蛋白原:90 mg/dl,无其他变化。腹部超声波检查报告为子宫肌瘤,尿检呈病理结果。妇科建议口服黄体酮。她开始接受抗生素治疗,输注红细胞、血浆和低温沉淀物,随后报告显示:因子 VII:2%,因子 IX:1%,因子 VIII:70%。她接受了因子 VII 重配剂(rFVII)治疗,出血得到缓解。医生给她开了预防性 rFVII 和血液监测处方。因急腹症再次入院,血红蛋白为 5 g/dl,凝血酶原时间(PT)/部分凝血活酶时间(PTT)延长,腹部断层扫描报告有腹腔积血。她接受了 rFVII 治疗,需要进行开腹手术和左侧输卵管切除术。随后再次入院,报告出现月经过多、hb6 g/dl、PT/PTT延长和两种凝血因子VII-IX,但在查阅的文献中未发现相关报告。
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Joint deficiency of coagulation factors VII and IX: a case report.

The diagnostic and therapeutic approach for an unusual clinical situation is presented. Twenty-three-year-old female patient is evaluated for hematuria and metrorrhagia. She reported irregular follow-up with hematology because of bleeding in childhood. She has also been receiving factor VII for 2 years, denying hospitalizations because of bleeding. Laboratory reported hb: 5.2 g/dl; platelets: 234 000/mm 3 ; PT: 100 s; PTT: 112 s, fibrinogen: 90 mg/dl without other alterations. Abdominal ultrasound reported uterine myoma, urinalysis was pathological. The gynecology indicated oral progesterone. She started antibiotic therapy, transfusion of red-blood cells, plasma, and cryoprecipitates and subsequently reported: factor VII: 2%, IX: 1% and VIII: 70%. She received factor VII-recombinant (rFVII), achieving resolution of bleeding. She was prescribed prophylactic rFVII and hematology monitoring. Readmission due to acute abdomen with Hb 5 g/dl, prolonged prothrombin time (PT)/partial thromboplastin time (PTT) and abdominal tomography reported hemoperitoneum. She received rFVII and required laparotomy and left oophorectomy. Then readmission to metrorrhagia, hb6 g/dl, prolonged PT/PTT and factor VII-IX of two coagulation factors were reported, without reports found in the literature consulted.

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来源期刊
CiteScore
1.90
自引率
0.00%
发文量
111
审稿时长
4-8 weeks
期刊介绍: Blood Coagulation & Fibrinolysis is an international fully refereed journal that features review and original research articles on all clinical, laboratory and experimental aspects of haemostasis and thrombosis. The journal is devoted to publishing significant developments worldwide in the field of blood coagulation, fibrinolysis, thrombosis, platelets and the kininogen-kinin system, as well as dealing with those aspects of blood rheology relevant to haemostasis and the effects of drugs on haemostatic components
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