获得性血友病A继发于SARS-CoV-2肺炎1例

IF 3.8 3区 医学 Q1 MEDICAL LABORATORY TECHNOLOGY Biochemia Medica Pub Date : 2022-10-01 DOI:10.11613/BM.2022.030801
Brkić Nikolina, Milić Marija, Bekavac Marija, Marković Maja, Perković Dubravka
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引用次数: 1

摘要

获得性血友病A (AHA)是一种危及生命的疾病。AHA的发病率极低,需要多学科的诊断和治疗方法。这是一例73岁男性病例报告,他表现为继发于严重急性呼吸综合征冠状病毒2 (SARS-CoV-2)肺炎的AHA。病人有广泛的皮肤出血和血肿。在凝血筛选试验中,活化的部分凝血活酶时间(APTT)与正常凝血酶原时间(PT)相比延长,这是进一步研究的指征。在与正常血浆的混合研究中,APTT不正确,因此怀疑是凝血因子抑制剂。随着出血的迹象,极低的因子VIII (FVIII)活性(2%)和FVIII抑制剂的存在,诊断为AHA并开始治疗。患者接受因子8抑制剂旁路剂(FEIBA)治疗3天,随后长期皮质类固醇和环磷酰胺治疗。排除了恶性和自身免疫性疾病是AHA最常见的病因。患者对治疗反应良好,APTT和FVIII活性逐渐正常化。据我们所知,本病例是第一例报道的SARS-CoV-2肺炎后新发AHA病例。如果患者有皮肤和粘膜出血,且没有个人和家族出血史,且有孤立的APTT,则应怀疑AHA的诊断。与临床实验室专家合作,调查任何孤立的APTT延长是很重要的。
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Acquired hemophilia A secondary to SARS-CoV-2 pneumonia: a case report.

The acquired hemophilia A (AHA) is a life-threatening condition. The incidence of AHA is extremely low, which requires a multidisciplinary approach to diagnosis and treatment. This is case report of 73-year-old man who presented with AHA secondary to severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) pneumonia. The patient had extensive skin bleeding and hematomas. In the coagulation screening tests activated partial thromboplastin time (APTT) was prolonged with normal prothrombin time (PT), which was indication for further investigation. The APTT in a mixing study with normal plasma did not correct so clotting factors inhibitors were suspected. With signs of bleeding, extremely low factor VIII (FVIII) activity (2%) and presence of FVIII inhibitors, AHA was diagnosed and treatment initiated. Patient was treated with factor eight inhibitor bypassing agent (FEIBA) for three days, followed by long-term corticosteroid and cyclophosphamide therapy. Malignant and autoimmune diseases as the most common causes of AHA were ruled out. The patient had a good response to therapy with gradual normalization of APTT and FVIII activity. To the best of our knowledge, the present case is the first reported case of de novo AHA after SARS-CoV-2 pneumonia. The diagnosis of AHA should be suspected in a patient with bleeding into the skin and mucous membranes without a previous personal and family history of bleeding, and with isolated prolonged APTT. It is important to investigate any isolated prolongation of APTT in cooperation with clinical laboratory experts.

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来源期刊
Biochemia Medica
Biochemia Medica 医学-医学实验技术
CiteScore
5.50
自引率
3.00%
发文量
70
审稿时长
>12 weeks
期刊介绍: Biochemia Medica is the official peer-reviewed journal of the Croatian Society of Medical Biochemistry and Laboratory Medicine. Journal provides a wide coverage of research in all aspects of clinical chemistry and laboratory medicine. Following categories fit into the scope of the Journal: general clinical chemistry, haematology and haemostasis, molecular diagnostics and endocrinology. Development, validation and verification of analytical techniques and methods applicable to clinical chemistry and laboratory medicine are welcome as well as studies dealing with laboratory organization, automation and quality control. Journal publishes on a regular basis educative preanalytical case reports (Preanalytical mysteries), articles dealing with applied biostatistics (Lessons in biostatistics) and research integrity (Research integrity corner).
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