Acquired Hemophilia A following Pfizer-BioNTech SARS CoV-2 mRNA vaccine, successfully treated with prednisolone and rituximab

Acquired Hemophilia A following Pfizer-BioNTech SARS CoV-2 mRNA vaccine, successfully treated with prednisolone and rituximab
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DOI:
10.1177/10781552221075545
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发表时间:
2022-01-28
影响因子:
1.3
通讯作者:
Mangos, Hilda M.
Mangos, Hilda M.
中科院分区:
医学4区
文献类型:
--
作者:
Murali, Aarya;Wong, Philip;Mangos, Hilda M.

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获得性血友病A(AHA)是一种罕见的出血性疾病,其特征是存在凝血因子VIII(FVIII)的自身抗体。AHA可以是特发性的,也可以发生在恶性肿瘤、自身免疫性疾病、药物或妊娠的背景下。最近,报告了COVID-19感染和疫苗接种后的AHA病例。病例报告我们报告的情况下,95岁的女性谁是免疫与辉瑞BioNTech SARS CoV-2 mRNA疫苗,剂量给予间隔三周。首次给药后一周,她的四肢出现自发性瘀伤,第二次给药后三周入院。检查发现右手背上有一个大血肿,并伴有出血和广泛的瘀斑。调查证实了AHA的诊断。治疗和结果初始治疗包括大剂量泼尼松龙,重组因子VIII和氨甲环酸。三天后没有明显的临床改善,因此开始静脉注射利妥昔单抗100 mg,每周一次,持续四周。两次给药后活化部分凝血活酶时间(aPTT)恢复正常,第+22天因子VIII水平达到0.68 U/ml。37天后患者成功出院。讨论已经记录了4例COVID mRNA疫苗(辉瑞和Moderna)接种后发生AHA的病例。在血小板计数正常的情况下,AHA应该是COVID-19疫苗接种后出现出血的患者的一个鉴别诊断。需要快速识别、及时启动免疫抑制治疗和严格的支持性护理,以最大限度地降低发病率和死亡率。
Introduction Acquired haemophilia A (AHA) is a rare bleeding disorder, characterised by the presence of autoantibodies to clotting factor VIII (FVIII). AHA can be idiopathic or occur in the context of malignancy, autoimmune disease, drugs, or pregnancy. Recently, cases of AHA following both COVID-19 infection and vaccination have been reported. Case report We report the case of a 95-year-old female who was immunised with the Pfizer-BioNTech SARS CoV-2 mRNA vaccine, with doses given three weeks apart. Spontaneous bruising over her extremities appeared one week after the initial dose, with hospital admission occurring three weeks after the second. Examination revealed a large haematoma on the dorsum of the right hand with resultant bleeding and widespread ecchymoses. Investigations confirmed a diagnosis of AHA. Management and outcome Initial management included high dose prednisolone, recombinant Factor VIII and tranexamic acid. There was no significant clinical improvement after three days, so intravenous rituximab 100 mg weekly for four weeks was commenced. The activated partial thromboplastin time (aPTT) normalised after two doses and Factor VIII level reached 0.68U/ml on day + 22. The patient was successfully discharged from hospital after 37 days. Discussion Four cases of AHA following administration of COVID mRNA vaccines (Pfizer and Moderna) have been documented. AHA should be a differential in patients presenting with bleeding following COVID-19 vaccination, in the presence of a normal platelet count. Rapid recognition, prompt initiation of immunosuppressive treatment and rigorous supportive cares are required to minimise morbidity and mortality.