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2.
BMJ Case Rep ; 15(3)2022 Mar 09.
Article in English | MEDLINE | ID: covidwho-1736044

ABSTRACT

Acquired haemophilia A (AHA) is a rare bleeding disorder with high morbidity and mortality, but it is eminently treatable if diagnosis and treatment are prompt. We report a case of AHA in Southeast Asia following the administration of the Pfizer-BioNTech COVID-19 vaccine. A man in his 80s developed multiple bruises 2 weeks after his first dose of the COVID-19 vaccine. Diagnosis was delayed due to his cognitive impairment and low clinical suspicion. This led to a representation with worsening ecchymosis, a left thigh haematoma and symptomatic anaemia. Laboratory testing was notable for an isolated prolongation of the activated partial thromboplastin time, which remained uncorrected in the mixing test. Further testing confirmed the presence of factor VIII (FVIII) inhibitors and low FVIII titres of 6.7%. He responded to treatment with intravenous methylprednisolone and recombinant activated FVII. Screening for autoimmune diseases and malignancies was negative.


Subject(s)
COVID-19 , Hemophilia A , Asia, Southeastern , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Factor VIII/therapeutic use , Hemophilia A/diagnosis , Hemophilia A/drug therapy , Hemophilia A/etiology , Humans , Male , SARS-CoV-2
3.
Thromb Res ; 211: 60-62, 2022 03.
Article in English | MEDLINE | ID: covidwho-1712998

ABSTRACT

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by neutralizing autoantibodies against coagulation Factor VIII. Immunomodulatory effects of SARS-CoV-2 vaccination are still poorly understood, with reports of immune-mediated conditions developing after immunization. In the province of Reggio Emilia, Northern Italy, we observed four cases of AHA following SARS-CoV-2 immunization with mRNA BNT162b2 vaccine (produced by Pfizer-BioNTech) during the first eight months from the beginning of SARS-CoV-2 vaccination campaign. During this time frame, 235,597 people received at least one dose of BNT162b2 vaccine. The total population of Reggio Emilia province is 526,349. The unusual observation of four cases of AHA in our province could be of interest and could sensitize healthcare personnel toward a possible complication of SARS-Cov-2 immunization. Nonetheless, vaccination benefits exceed potential side effects and play a central role in individual and public health to effectively protect people from COVID-19 and to stop the pandemic.


Subject(s)
COVID-19 , Hemophilia A , /adverse effects , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Humans , RNA, Messenger , SARS-CoV-2 , Vaccination/adverse effects
4.
Haemophilia ; 28(2): 247-253, 2022 Mar.
Article in English | MEDLINE | ID: covidwho-1685308

ABSTRACT

INTRODUCTION: The haemophilia community on Twitter is diverse, consisting of advocacy groups, patients, physicians, researchers and other users. However, the scope of this community is uncharacterized, and limited data is available regarding effective participation in this community. AIM: To assess the types of users active in the haemophilia community on Twitter, as well as major themes present in haemophilia-related tweets. METHODS: Forty-nine thousand five hundred and twelve tweets between September 2019 and September 2021 were classified using regular expressions. A subset of the classified tweets was manually analysed to identify prevalent discussion themes. RESULTS: Among the top 250 users by post count, the largest categories of users were support and advocacy groups, people with bleeding disorders and healthcare providers. The largest thematic categories of tweets were gene therapy, contaminated haemophilia blood products, haemophilia research, clinical management of haemophilia and COVID-19. While misinformation was rare, negative and incorrect perceptions of haemophilia were present among the general public. CONCLUSION: Our results demonstrate patterns of effective Twitter usage for patient care, research and advocacy purposes among the haemophilia community.


Subject(s)
COVID-19 , Hemophilia A , Social Media , Communication , Hemophilia A/therapy , Humans , SARS-CoV-2
7.
BMJ Case Rep ; 14(10)2021 Oct 01.
Article in English | MEDLINE | ID: covidwho-1447986

ABSTRACT

An 80-year-old man with no personal or family history of bleeding, presented to hospital with extensive haematomas and skin bruising after using doxycycline. His basic lab workup was concerning for a coagulopathy with an elevated activated partial thromboplastin time and significant anaemia. Mixing studies and other factor levels were tested that led to the diagnosis of acquired haemophilia A with low factor VIII levels and high factor VIII antibodies. He was started on steroids, but his haemoglobin level continued to drop. Later, during his treatment, he was given multiple therapeutic agents, including cyclophosphamide, rituximab and recombinant factor VII (NovoSeven-R). Gradually factor VIII levels increased and haemoglobin stabilised. The hospital course was complicated by COVID-19 pneumonia leading to acute respiratory distress syndrome; the patient eventually expired due to respiratory failure.


Subject(s)
COVID-19 , Hemophilia A , Aged, 80 and over , Doxycycline/therapeutic use , Hemophilia A/chemically induced , Hemophilia A/diagnosis , Hemophilia A/drug therapy , Humans , Male , Partial Thromboplastin Time , SARS-CoV-2
8.
BMJ Case Rep ; 14(7)2021 Jul 20.
Article in English | MEDLINE | ID: covidwho-1388480

ABSTRACT

A 65-year-old man presented with symptoms of severe subcutaneous bleeding in his arm, which led to compartment syndrome requiring fasciotomy and massive blood transfusion protocol. Medical history was significant for history of autoimmune thyroid disease. Workup revealed elevated partial thromboplastin time, decreased factor VIII levels and elevated factor VIII inhibitor levels. He was worked up for causes of acquired haemophilia A and was found to have an elevated SARS-CoV-2 antibody level. Given his negative workup for other secondary aetiologies, we suspect that the cause of his haemophilia A was from his SARS-CoV-2 infection, which has been observed previously in various case reports.


Subject(s)
COVID-19 , Hemophilia A , Aged , Hemophilia A/complications , Hemophilia A/diagnosis , Humans , Male , Partial Thromboplastin Time , SARS-CoV-2
12.
Haemophilia ; 27(5): 736-743, 2021 Sep.
Article in English | MEDLINE | ID: covidwho-1288288

ABSTRACT

BACKGROUND: Emicizumab, a bispecific monoclonal antibody administered subcutaneously, mimicking the action of activated coagulation factor VIII, has been approved in Europe for use in patients with severe hemophilia of all ages. AIMS: To assess availability, acceptance, adverse events, efficacy and laboratory monitoring of emicizumab and the effect of the coronavirus disease 2019 (COVID-19) pandemic on its use. METHODS: Online questionnaire sent to 144 hemophilia treatment centres (November 2020 to January 2021). RESULTS: Forty-six physicians from 21 countries responded, with a total of 3420 patients with severe HA under their care. Emicizumab was widely available, for 100% of inhibitor patients and 88% of non-inhibitor patients. No major adverse events were reported. Four reported deaths in patients on emicizumab were not thought to be related to emicizumab. An annualized bleeding rate (ABR) of zero was achieved in 73% of inhibitors patients. Haemostasis was satisfactory in the majority of minor (93.7%) and major (90.7%) surgical procedures performed while on emicizumab. Inhibitor titers were monitored in 78.4% of inhibitor patients on emicizumab, but chromogenic FVIII assay was only available in 73% of centres. The COVID-19 pandemic did not have a major impact on the adoption of emicizumab in most centres (64.9%). CONCLUSION: Three years after its rollout in Europe, emicizumab is widely available. Clinical efficacy and safety were evaluated to be very good, keeping in mind the inherent limitations of the study. Unmet needs include establishment of treatment guidelines for surgery and breakthrough bleeding, limited expertise, especially in young children, and availability of laboratory assays.


Subject(s)
Antibodies, Bispecific , Antibodies, Monoclonal, Humanized , Hemophilia A , Antibodies, Bispecific/therapeutic use , Antibodies, Monoclonal, Humanized/therapeutic use , COVID-19 , Europe , Factor VIII , Hemophilia A/drug therapy , Humans , Pandemics , Surveys and Questionnaires
14.
J Thromb Haemost ; 19(6): 1515-1518, 2021 06.
Article in English | MEDLINE | ID: covidwho-1238450

ABSTRACT

Acquires hemophilia A (AHA) is rare bleeding condition commonly associated with malignancy, autoimmune disease, or pregnancy. We report a case of a 69-year-old gentleman who developed bleeding symptoms after receiving COVID-19 vaccine. Laboratory testing showed isolated prolongation of the activated partial thromboplastin time, and normal von Willebrand factor. Further testing confirmed the presence of factor VIII inhibitor. To date, no cases of AHA have been reported after exposure to COVID-19 vaccine. There have been two cases of AHA following seasonal flu and H1N1 vaccination, as well as two cases of AHA following COVID-19 infection. We present a summary of these cases and review of literature of autoimmune reactions following vaccination.


Subject(s)
COVID-19 , Hemophilia A , Influenza A Virus, H1N1 Subtype , Aged , COVID-19 Vaccines , Factor VIII , Female , Hemophilia A/diagnosis , Humans , Male , Pregnancy , SARS-CoV-2
16.
Nat Rev Drug Discov ; 20(3): 170-171, 2021 03.
Article in English | MEDLINE | ID: covidwho-1132041
18.
Haemophilia ; 27(1): 41-48, 2021 Jan.
Article in English | MEDLINE | ID: covidwho-1066683

ABSTRACT

INTRODUCTION: The SARS-CoV-2 coronavirus-induced infection (COVID-19) can be associated with a coagulopathy mainly responsible for pulmonary microvasculature thrombosis and systemic thromboembolic manifestations. The pathophysiology and management of the COVID-19 coagulopathy are likely more complex in patients with inherited bleeding diseases such as haemophilia. These individuals might indeed present with both bleeding and thrombotic complications and require simultaneous antithrombotic and haemostatic treatments. OBJECTIVE: We propose practical guidance for the diagnosis and management of COVID-19 coagulopathy in persons with haemophilia. RESULTS: Continuation of regular haemostatic treatment is recommended for ambulatory patients. For patients requiring hospital admission and on replacement therapy with factors VIII or IX concentrates, prophylaxis with concentrates should be intensified according to the risk of bleeding complications and associated with prophylactic doses of LMWH. For patients on nonreplacement therapy, emicizumab should be continued and possibly combined with factor VIII and prophylactic doses of LMWH depending on the risk of bleeding and thrombosis. Dose escalation of LMWH tailored to the risk of thrombosis can be employed but not supported by evidence. CONCLUSIONS: These practical recommendations are based on the current literature on COVID-19 with its impact on haemostasis, indications and modalities for thromboprophylaxis mainly in nonhaemophilic patients and how that is likely to affect persons with haemophilia in different circumstances. They will need to be tailored to each patient's clinical status and validated in future studies.


Subject(s)
COVID-19/complications , Disseminated Intravascular Coagulation/complications , Hemophilia A/complications , Antibodies, Bispecific/therapeutic use , Antibodies, Monoclonal, Humanized/therapeutic use , Anticoagulants/therapeutic use , COVID-19/diagnosis , COVID-19/therapy , Disease Management , Disseminated Intravascular Coagulation/diagnosis , Disseminated Intravascular Coagulation/therapy , Factor IX/therapeutic use , Factor VIII/therapeutic use , Hemophilia A/diagnosis , Hemophilia A/therapy , Heparin, Low-Molecular-Weight/therapeutic use , Humans
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