ABSTRACT
Background : Severe acute respiratory syndrome coronavirus 2 (SARS-CoV 2) infection is a systemic disease with a pulmonary prognosis. Alsace, in northern France was one of the first hotspot for Covid-19. Aims : Among the patients with hemorrhagic congenital disease (HCD), data about its incidence are scarce. Methods : We retrospectively report cases of Covid-19 in the Regional heamophilia treatment Center of Strasbourg in France since the beginning of the SARS-CoV 2 outbreak. Results : Among the 620 patients followed, we retrieved 26 biologically confirmed cases of Covid-19: 18 diagnosed with a positive PCR-based viral RNA detection from nasopharyngeal swabs and 8 with serological testing. We found 14 hemophilia's patients, 1 factor XI's severe deficiency, 6 patients with thrombopathy and 5 Willebrand diseases. Demographic characteristics are listed in Table 1. Half of the infection occurred in the spring 2020. Nine patients were asymptomatic. No specific antiviral treatment were used. Two patients were hospitalized (10 and 90 days) including one in intensive care. The last one exhibited an acute respiratory distress syndrome and needed for mechanical ventilation. Despite his congenital thrombopathy, he required curative anticoagulation because of a pulmonary embolism. A significant activation of coagulation was also found in a young woman with Glanzmann ' s thrombasthenia without clinically proven thromboembolism disease. The two patients featured a major hemorrhagic syndrome (menorrhagia and hematuria). This condition increased complexity in their management, with the need for platelet transfusion. The outcome was favorable for all cases. Conclusions : Coagulation abnormalities and the reported high frequency of venous thromboembolism during SARS CoV 2 infection makes the management of patients with HCD trickier. It would be interesting to collect cases of Covid-19 in patients with HCD on a national and international level.