Acquired Haemophilia associated with SARS-CoV2 infection

We present the case of a 73-year-old man, with a history of SARS-CoV2 infection ( January 2021), who came to the emergency department three months post infection, with complaints of left hip and knee pain, that turned out to be a substantial thigh hematoma. Analysis showed a normocytic/normochromic anaemia (9,0 g/dL), prolonged aPTT (63.2 seconds;normal range 24.7-39.0 sec.), with normal prothrombin time. We arrived at a diagnosis of Acquired Haemophilia A. Treatment was promptly started, with clinical and laboratory improvement. After the vaccination to SARS-CoV2, a relapse was observed. Acquired Haemophilia A is a rare, autoimmune disease, distinguished by the presence of inhibitors against factor VIII. It's characterised by subcutaneous hematomas and muscle bleeding, with prolonged aPTT. SARS-CoV2 infection has already been mentioned as a possible cause.

Abnormal postpartum uterine bleeding in adolescence associated with SARS-CoV-2 infection

Background: With a prevalence of 1-3 cases per million, acquired haemophilia A (AHA) is a rare autoimmune bleeding disorder caused by the presence of neutralizing antibodies against factor VIII. Even though diagnosis of this bleeding disorder is rarely established among children and adolescents, AHA may lead to severe, life-threatening hemorrhage in this age group, and therefore it requires special caution. Case report: 19 year old primigravida with confirmed SARS-CoV-2 infection was admitted to hospital due to prolonged vaginal bleeding six weeks postpartum. All gynaecological causes of uterine bleeding were excluded, Foley catheter was placed, but the bleeding still persisted. Coagulation tests revealed isolated deranged aPTT values. Further haematology evaluation demonstrated factor VIII deficiency, presence of factor VIII inhibiting factors, and the diagnosis of AHA was proposed. The anti-inhibitor coagulant complex drug was introduced and patient has responded positively to the treatment. Conclusion(s): Due to disturbance of immune system, pregnancy and postpartum period represent predilection time for AHA development. Furthermore, viral infection in pregnancy, such as COVID-19, might be considered as an additional risk factor for AHA development and several reported cases of AHA after COVID-19 infection support this hypothesis. Even though AHA is a rare disease, due to its high mortality rate of more than 20%, it should be considered in all cases of unusual bleeding of unknown cause in all age groups. Publication of this case report is approved by Institutional Review Board.Copyright © 2023

A cross-sectional follow-up study of physical activity in adults with moderate and severe haemophilia.

AIM: To conduct a cross-sectional follow-up assessment of physical activity (PA) in people with moderate and severe haemophilia (PwMSH) from the Irish Personalised Approach to the Treatment of Haemophilia (iPATH) study. METHODS: Between June-December 2021, participants' PA was measured over one week using accelerometery, and was compared with their previously measured data from the original iPATH assessment. Self-awareness of PA and the impact of the Covid-19 pandemic on PA, pain, mobility and function were retrospectively examined using a survey. RESULTS: Of 30 participants who returned surveys [n = 19, severe (FVIII, <.01 IU/mL); n = 4, moderate (FVIII, .01-.05 IU/mL); n = 7, severe (FIX, <.01 IU/mL); age: 47 (36, 55) years], 28 completed accelerometery (follow-up time: 3 years). There were no significant differences in accelerometer PA (all p > .05), but achievement of World Health Organisation guidelines increased (67.9%-75.0%; p = .646). Increased self-awareness of PA was reported by 76.7%, and 66.7% reported desires to become more physically active. Compared to normal, most reported either no differences or lower levels of PA during lockdown restrictions. Self-reported PA increased for most when restrictions eased from April 2021 onwards. Beyond the pandemic, concerns included pain and access to exercise resources. CONCLUSION: Self-reported PA throughout the pandemic was variable, whilst there were no significant differences in objectively measured PA between assessment periods, despite reports of increased self-awareness and desires to be physically active at follow-up. Further qualitative research is needed to design personalised PA and health interventions, capturing perspectives of patients, their families, and multi-disciplinary haemophilia healthcare providers.

Management of acquired haemophilia A in severe Covid-19: Haemostatic bridging with emicizumab to keep the balance between bleeding and thrombosis.

Acquired haemophilia A (AHA) is an autoimmune bleeding disorder caused by autoantibodies blocking coagulation factor VIII (FVIII). Haemostatic management of AHA and concomitant thrombotic risk is difficult. We cover the management of a 75-year-old male with severe Covid-19, a prothrombotic disease, and de novo AHA with severe muscle bleeding, a disease requiring highly thrombogenic haemostatic therapy and immunosuppression-a challenging combination. FVIII activity was measured using human and bovine reagents to differentiate between endo- and exogenous FVIII activity. For haemostatic control, recombinant human activated FVII was given, followed by emicizumab, as a less thrombogenic long-term haemostatic agent. Steroids were used as initial immunosuppressive therapy. Later, rituximab was used for inhibitor eradication. No thromboembolic events occurred, and bleeding was effectively controlled. Emicizumab achieved haemostatic balance in a patient under haemorrhagic and thrombogenic conditions. Individual risk assessment is needed to guide treatment decisions in patients threatened by simultaneous bleeding and thrombosis.

Total Hip Replacement in Severe Haemophilia A: Challenges and Feasibility.

The management of haemophilic patients is complicated due to multiple comorbidities. A dedicated haemophilia treatment centre with a multidisciplinary team can plan and execute elective orthopaedic surgery in such haemophilic individuals. A cementless total hip arthroplasty (THA) was performed in a 26-year-old male patient with haemophilic arthropathy of the right hip under factor VIII replacement therapy based on activated partial thromboplastin time levels. The patient received a preoperative dose of recombinant anti-haemophilic factor. Venous thromboembolic event prophylaxis was not given. Postoperative radiographs demonstrated successful prosthesis placement. The patient could bear weight and walk unassisted two weeks after surgery. THA in patients with haemophilia leads to significant improvement in joint function with a relatively low incidence of complications with modern techniques and haematological management.

Case Report: Acquired Haemophilia A Following mRNA-1273 Booster Vaccination Against SARS-CoV-2 With Concurrent Diagnosis of Pleomorphic Dermal Sarcoma.

While the global pandemic caused by severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) is still ongoing and new virus variants are emerging, there is a universal need for vaccines to protect individuals from severe complications and ideally control the pandemic by enabling herd immunity. Several vaccines against SARS-CoV-2 have been approved and are widely used to stem the recurring waves of coronavirus disease 2019 (COVID-19). Post-marketing surveillance is essential to record even rare safety issues related to these new vaccines. Among these issues, several autoimmune phenomena have been recorded in temporal association with and feasibly triggered by a vaccination. Acquired haemophilia A (AHA) is a rare condition characterized by new-onset haemorrhagic diathesis caused by an inhibitor of blood clotting factor VIII (FVIII), often in the elderly and most commonly associated with autoimmune or malignant disease. There have been a small number of AHA cases triggered by vaccinations, including those against SARS-CoV-2. We report the first case of AHA in temporal association with an mRNA-1273 booster vaccination. The diagnosis was made promptly, and the patient received appropriate care including immunosuppression using glucocorticoids, cyclophosphamide (CYC) and rituximab (RTX). The haemorrhage ceased after escalation of treatment, and the patient is recovering. Concurrent malignancy was initially ruled out using a wide scope of diagnostic tests, but pleomorphic dermal sarcoma (PDS) of the forehead occurred after initiation of specific AHA immunosuppressive treatment. Since large vaccination programs are ongoing worldwide and potential adverse events during post-marketing surveillance have been reported following vaccination against SARS-CoV-2, this case illustrates challenges in rare events occurring in association with SARS-CoV-2 vaccination and to proof a causal relationship. Therefore, there is an urgent need for reporting any events in association with SARS-CoV-2 vaccination, but also a crucial discussion about possible concurrent triggers and follow-up information about individual patients.

Anticoagulation for Stroke Prevention after Restoration of Haemostasis with Emicizumab in Acquired Haemophilia A.

Acquired haemophilia A (AHA) is a rare haemorrhagic disorder caused by the development of autoantibodies inhibiting factor VIII function. It predominantly affects the elderly, who are often burdened with a considerable number of comorbidities, and can result in life-threatening bleeding. The management of AHA consists of two aspects: inhibitor eradication with an immunomodulator and bleed control with a bypassing agent. Here we present a case of AHA with a high titre inhibitor in a patient with extensive comorbidities and atrial fibrillation in whom inhibitor eradication could not be achieved within a few weeks using corticosteroids alone. Due to coronavirus disease (COVID)-19 restrictions and complications of care, emicizumab offered an effective and convenient therapy, not only sparing the need for continued and intensified inhibitor eradication, but also allowing anticoagulation for stroke prophylaxis. LEARNING POINTS: Emicizumab may offer a suitable option for bleeding prophylaxis when inhibitor eradication is not achievable with immunotolerance treatment, especially in the age of the COVID-19 pandemic when the consequences of immunosuppression can be detrimental.Bleeding prophylaxis with emicizumab may enable long-term anticoagulation in patients with acquired haemophilia A during inhibitor eradication.The prothrombotic risks of emicizumab are not yet sufficiently characterized.

Registry of patients with congenital bleeding disorders and COVID-19 in Madrid.

INTRODUCTION: We present the first registry of patients with congenital bleeding disorders and COVID-19. The study has been carried out in the Community of Madrid, which has the highest number of cases in Spain. The objective is to understand the incidence of COVID-19, the course of the disease if it occurs and the psychosocial and occupational impact on this population. METHODS: We included 345 patients (246 of haemophilia, 69 of von Willebrand Disease, two rare bleeding disorders and 28 carriers of haemophilia). A telephone survey was used to collect the data. RESULTS: Forty-two patients presented symptoms suggestive of infection by COVID-19, and in six cases, the disease was confirmed by RT-PCR. The cumulative incidence of our series was 1.73%. It is worth noting the complexity of the management of COVID-19 in two patients on prophylaxis with non-factor replacement therapy. Adherence to the prescribed treatment was maintained by 95.5% of patients. Although 94% were independent for daily living activities, 42.4% had a recognized disability and 58% required assistance, provided by the Madrid Haemophilia Association (Ashemadrid) in 75% of cases. Only 4.4% of consultations were held in person. CONCLUSIONS: Patients with congenital bleeding disorders infected with SARS-CoV-2 presented a mild course of the disease that did not require admission. Their identification and treatment by a specialist team from a Haemophilia Treatment Center are essential to make a correct assessment of the risk of haemorrhage/thrombosis. COVID-19 had a major impact on the psychosocial aspects of these patients which must be remedied with recovery plans.

How haemophilia A impacts severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2) treatment: a case report.

The typical symptoms of COVID-19 mimic those of the common season flu. In addition, several changes in the coagulation processes have been observed. To date, it's not fully clear how COVID-19 may affect patients with hereditary bleeding disorders. Anticoagulation in patients with haemophilia is still debated, but in this case could be needed. We are reporting a case of an elderly patient with mild haemophilia A hospitalized for Sars-Cov-2. On the 15th day of hospitalization, we observed an increase of all coagulation parameters. An antithrombotic prophylaxis at low dosage was immediately started, then increased at prophylactic dosage. Even if much more data are needed to ascertain the real thrombotic risk of haemophilia A in COVID-19 patients, it's clear that the FVIII and vWF should be strictly monitored in order to promptly establish an adequate treatment and avoid the onset of thromboembolic events, even fatal, causing many deaths in COVID-19 patients.