Knee replacement surgery in a patient with acquired von Willebrand disease: a case study with recommendations for patient management.

Introduction and importance: Acquired von Willebrand disease (AvWD) is a rare underdiagnosed bleeding disorder caused by alterations in the levels of the major blood-clotting protein von Willebrand factor (vWF). The clinical and laboratory parameters of AvWD are similar to congenital vWD, but it is found in individuals with no positive family history with no underlying genetic basis. The disease remains multifactorial and incompletely understood. Proposed mechanisms include the development of autoantibodies to vWF, absorption of high molecular weight vWF multimers that impair normal function, shear stress induced vWF cleavage and increased proteolysis.The aetiology of the disease is variable, the most common being hematoproliferation, lymophoproliferation, myeloproliferation and autoimmune and cardiovascular disorders. Consensus and protocols for AvWD patients that require major surgery are currently lacking. Patients with AvWD can experience thrombotic events during surgery as a result of therapeutic interactions with pro-thrombotic risk factors. Case presentation: Here, the authors report a patient with AvWD requiring a knee prosthesis implantation due to chronic pain, limited range of motion and functional impairment. The patient had a high risk of bleeding during surgery and was at risk of thrombosis due to age and obesity. Clinical discussion: Perioperative care required a collaborative approach and the management of bleeding. The patient was administered vWF concentrate Willfact lacking Factor VIII to prevent haemorrhage and to minimize the risk of thrombosis. Conclusion: The treatment was effective and well-tolerated. The authors use this information to provide recommendations for AvWD patients for whom major surgery is indicated.

Risk factors determining central venous access device-associated deep vein thrombosis resolution in children: a retrospective study.

The risk factors associated with the probability of central venous access device (CVAD)-associated deep vein thrombosis (DVT) resolution have been hardly evaluated in children. Current guidelines suggest anticoagulation for a maximum of 3 months in patients with provoked DVT if the provoking factor is resolved. To know if the thrombus will resolve after anticoagulant therapy will help to choose whether to initiate and/or continue this treatment or not. We did a retrospective study of 85 pediatric patients (45 girls, 40 boys) with CVAD-associated DVT to examine the risk factors associated with lack of thrombus resolution in the first 6 months after diagnosis. Sixty-two children had their thrombosis resolved after a median of 50 days (p25-p75 25-97) since diagnosis. In multivariate analysis, variables significantly associated with no resolution were total occlusion (OR 12.50, 95% CI 2.99-52.14, p=.001), location in upper extremity, head, and neck veins (OR 17.70, 95% CI 1.64-191.43, p=.018); collateral circulation in the first 45 days after diagnosis (OR 33.55, 95% CI 2.42-464.71, p=.009); and having between 0 and 3 prothrombotic risk factors at diagnosis (OR 6.20, 95% CI 1.21-31.75, p=.029).Conclusion: CVAD-associated DVT resolution in children in the first 6 months since diagnosis was significantly lower if the thrombosis was occlusive, if it was located in the upper extremity, head, and neck veins; if collateral circulation was seen on ultrasound in the first 45 days; and/or when the patient showed less prothrombotic risk factors at diagnosis. What is Known: • The risk factors associated with central venous access device-associated deep vein thrombosis resolution have been hardly evaluated in children. • Current guidelines suggest anticoagulation for a maximum of 3 months in provoked vein thrombosis if the provoking factor is resolved. What is New: • Thrombus resolution was lower if it was occlusive, located in the upper extremity veins, if collateral circulation was seen, and with less prothrombotic risk factors at diagnosis. • To know if the thrombus will resolve after anticoagulation will help to choose whether to initiate and/or continue it or not.

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.