--%>
登录/注册    
血友知识
当前位置:首页 > 血友知识 > 什么是血友病?
血友知识
Blood friend knowledge
    患者登记
Registration
    志愿者登记
volunteers register
    我要捐款
    我要提问
    救助项目
    救助项目
联系我们
地 址:北京市石景山区八角北路社区
42号楼3单元37号
电 话:010-59425987
     13051099010
     邮 箱:hhc@xueyou.org

COVID-19对出血性疾病群体的特定风险

详细介绍:

WFH COVID-19工作团队: Assad Haffar, Cedric Hermans, Barbara Konkle, Brian O’Mahony, David Page, Flora Peyvandi, Steve Pipe, Mark Skinner and Radek Kaczmarek and Glenn Pierce, Co-chairs


原文翻译:刘国青



一、感染SARS-CoV-2(病毒)和COVID-19(疾病)的风险


1.在免疫功能正常的出血性疾病患者中,未发现对感染的敏感性增加。SARS-CoV-2主要通过感染者呼出空气中的飞沫传播。这些飞沫进入上呼吸道并在此感染。


2.目前没有相关信息表明HIV携带者感染该疾病的风险增加。但是,一旦被感染,免疫力低下的人患重症的风险就会高得多。对于艾滋病毒,包括:


(1)CD4 T细胞计数低的人(例如,<200)

(2)没有接受抗逆转录病毒治疗的人

(3)如果还有其他与重症COVID-19相关的潜在疾病



二、如果患有COVID-19感染和出血性疾病的风险


1.这是一种可以引起多种疾病的潜在致命感染,从无症状到重症肺炎和致命的系统性的后遗症。年龄较大的人和存在确定危险因素的人罹患重症和致死性疾病的风险更大,同时儿童和年轻人也可能发展为重症疾病,尽管发生频率较低。


2.危险因素包括其他系统性疾病:高血压,糖尿病,心血管疾病和免疫抑制。


3.高血压患者不应该停止服药。目前的证据不支持改变高血压的管理方式。


4.随着COVID-19的进展,凝血途径被激活做为宿主控制病毒感染的炎症反应的一部分。具体而言,D-二聚体是纤维蛋白在血凝块中降解时的产物,在许多住院的COVID-19患者体内升高。D-二聚体是血凝块(血栓)形成和分解的指标。


5.许多重症COVID-19患者可能存在与高致死率相关的明确的弥散性血管内凝血(DIC)。DIC是一种可能由对病毒以及感染导致的受损组织引起的全身性炎症反应相关的凝血障碍。在DIC中,观察到血小板减少、凝血筛查时间延长(PT和aPTT)以及纤维蛋白原减少。


(1)建议所有存在DIC体征或症状的个体都密切监测出血和血栓形成情况。


(2)对于D-二聚体水平升高和重症感染的患者,建议将抗凝剂(例如低分子量肝素,LMWH)作为治疗方案的一部分。使用抗凝剂应同时应用因子替代治疗。


(3)如果血友病患者发生血栓事件,则详细的报告很重要(包括COVID状态,实验室评估,影像学,替代治疗方式)。



6.如果确诊COVID-19,应继续因子替代治疗中的预防治疗,并且如果因严重感染而住院治疗,则可能需要像治疗重大创伤一样的较高因子谷浓度水平。


7.对于正在应用非因子替代治疗如艾美赛珠或其他研究药物(如fitusiran,抗TFPI))的血友病患者合并COVID-19感染之后发生血栓并发症的风险尚不清楚。


(1)对于应用艾美赛珠的血友病A患者,尚不清楚该药物如何与由感染引起的凝血障碍相互作用,故建议密切监测血栓形成情况。


(2)应坚持预防治疗,在一些漏服剂量的情况,长半衰期(约30天)的艾美赛珠由于它能较长时间保持活性,故应考虑应用。


(3)应该对患者进行评估以确定他们是否需要另加用凝血因子替代治疗。


8.可以按照推荐的治疗方案考虑使用抗凝剂。


9. 由于已知艾美赛珠与aPCC之间的药物相互作用,对于存在FVIII抑制物接受艾美赛珠治疗的患者,如果患者需要应用aPCC,则应采取额外的预防措施。


10.需要注意在应用艾美赛珠的患者中,某些一期法测凝血功能的试验(如通常用于诊断和监测DIC患者的aPTT)会高估了患者的凝血功能,因此可能掩盖了其本来存在的凝血障碍。


11.建议研究人员从研究赞助商和医学监测者那里寻求针对这些药物临床试验计划中受试者的指导。推荐患者应告知医疗保健提供者他们正在临床研究中并请其血液学专家参考。


12.对于参加基因疗法临床试验的患者,除了在《血友病患者的实用建议》中概述的免疫抑制时注意感染风险的警告外,对基因治疗反应欠佳的患者中还应考虑补充更高的凝血因子水平(例如像治疗重大创伤)。


13.患所有严重程度的出血性疾病和COVID-19的患者应有资格根据其病情要求使用所有可用的疗法(例如通气支持,ECMO,血液滤过)。


(1)患有血友病不应成为COVID-19的侵入性治疗的排除个体。




10d95522c9f3f201747bb0e1704d285.jpg


WFH COVID-19 Task Force: Assad Haffar, Cedric Hermans, Barbara Konkle, Brian O’Mahony, David Page, Flora Peyvandi, Steve Pipe, Mark Skinner and Radek Kaczmarek and Glenn Pierce, Co-chairs

Risks of acquiring SARS-CoV-2 (the virus) and COVID-19 (the disease)

  • No increased susceptibility to infection has been found in immunocompetent patients with bleeding disorders. SARS-CoV-2 is passed primarily through droplets in the air coming from infected persons. These droplets get into the upper respiratory tract where they establish an infection.

  • https://www.who.int/news-room/commentaries/detail/modes-of-transmission-of-virus-causing-covid-19-implications-for-ipc-precaution-recommendations

  • There is no information about whether persons with HIV are at increased risk of acquiring the infection. However, if infected, immunocompromised people are at much higher risk for severe disease. For HIV, that includes:


  • People with a low CD4 T-cell count (e.g.,<200)

  • People not on antiretroviral HIV treatment

  • If there are other underlying diseases associated with severe COVID-19

  • https://www.cdc.gov/coronavirus/2019-ncov/need-extra-precautions/hiv.html

  • Risks if you have COVID-19 infection and a bleeding disorder


  • This is a potentially deadly infection that causes a spectrum of disease, from asymptomatic to severe pneumonia and lethal systemic sequelae. While older individuals and those with identified risk factors are at greater risk of serious and lethal disease, children and young adults may also develop severe disease, although less frequently.

    • https://www.cdc.gov/coronavirus/2019-ncov/need-extra-precautions/people-at-higher-risk.html

  • Risk factors include other systemic diseases including hypertension, diabetes, cardiovascular disease, and immunosuppression.

  • Individuals with hypertension should not discontinue their medication. Current evidence does not support changes in the management of hypertension.

  • As COVID-19 progresses, coagulation pathways are activated as part of the host inflammatory response to limit the viral infection. Specifically, D-dimers, products of fibrin as it is degraded within clots, are elevated in many cases of hospitalized COVID-19 patients. D-dimers are an indicator of clot (thrombus) formation and breakdown.

  • More severe COVID-19 disease may lead to overt disseminated intravascular coagulation (DIC), associated with high mortality. DIC is a coagulopathy that may arise from the systemic inflammatory response to the virus and damaged tissue caused by the infection. In DIC, decreased platelets, prolonged screening tests (PT and aPTT), and decreased fibrinogen are observed.

    • Close monitoring for bleeding and thrombosis is recommended for all individuals who progress with signs or symptoms of DIC.

    • Anticoagulants (e.g., low molecular weight heparin, LMWH) are being recommended as part of treatment protocols for patients with elevated D-dimers and severe infection. Use of anticoagulants should be accompanied by factor replacement therapy.

    • Should a thrombotic event occur in a hemophilia patient, detailed reporting is important (including COVID-status, laboratory assessments, imaging, replacement therapy).

  • If COVID-19 is diagnosed, prophylaxis with factor replacement therapy should be continued, and if hospitalized for severe infection, higher trough levels may need to be considered as if treating major trauma.

  • The risk of thrombotic complications for hemophilia patients who are currently treated with non-factor replacement therapies including emicizumab or other investigational agents (e.g. fitusiran, anti-TFPI) is unknown in the presence of COVID-19 infection.

    • In individuals with hemophilia A receiving emicizumab, it is unknown how the drug may interact with coagulopathy caused by infection and close monitoring for thrombosis is recommended.

    • Prophylaxis should be continued, and in the event of missed doses, the long half-life (~30 days) of emicizumab should be taken into consideration, since it will be present and active for a prolonged period of time.

    • Patients should be assessed to determine if they need additional clotting factor replacement therapy.

  • Anticoagulants may be considered as per recommended treatment protocols.

  • In patients with FVIII inhibitors receiving emicizumab, extra precautions should be taken if a patient requires aPCC due to the known drug-drug interaction between emicizumab and aPCC.

    • https://www.hemlibra.com/hcp/safety.html?c=hea165155ea50d&gclid=EAIaIQobChMI7sT20bvG6AIVlddkCh3lFAG0EAAYASACEgI-2vD_BwE&gclsrc=aw.ds

  • Be aware that some one-stage coagulation assays, such as aPTT which is often used to diagnose and monitor patients in DIC, overestimate coagulation in patients on emicizumab and thus may mask coagulopathy.

  • Investigators are advised to seek guidance from the study sponsors and medical monitors for subjects within clinical trial programs for these agents. Patients should inform health care providers they are on a clinical study and reference back to their hematologist is recommended.

  • For patients participating in a clinical trial of gene therapy, in addition to caution regarding infection risk when immunosuppressed, as outlined in Practical Recommendations for People with Hemophilia https://news.wfh.org/covid-19-coronavirus-disease-2019-pandemic-caused-by-sars-cov-2-practical-recommendations-for-hemophilia-patients/, supplementation to higher coagulation factor levels (e.g. as if treating major trauma) could be considered in those who have had a suboptimal response to the gene therapy.

  • Patients with bleeding disorders of all severities and COVID-19 should be eligible for all available therapies that would be required depending on their condition (e.g., ventilation support, ECMO, hemofiltration).

  • Having hemophilia should not exclude individuals from invasive management of COVID-19.


上一条】 【返回列表】 【下一条
关于我们
血友知识
登记交流
联系方式
电 话:010-59425987
13051099010
E-mail:hhc@xueyou.org
地址:北京市石景山区八角北路42号楼3单元37号
通讯地址:北京市石景山区苹果园街道八大处高科技园区西井路3号崇新大厦3号楼3405室
Copyright 1996- 2014 xueyou.org 血友之家 版权所有 京ICP备05063694号-1
技术支持:博搜网络