key: cord-0791726-3l3ah3o6 authors: Dorgalaleh, Akbar; Tabibian, Shadi; Mohammadamini, Mahboobeh; Bahraini, Mehran; Dabbagh, Ali; Noroozi‐Aghideh, Ali; Shams, Mahmood; Anvar, Ali; Namvar, Ali; Baghaipour, Mohammad Reza; Rad, Fariba; Azadi, Parviz title: Do congenital bleeding disorders have a protective effect against COVID‐19? A prospective study date: 2020-11-30 journal: Int J Lab Hematol DOI: 10.1111/ijlh.13413 sha: 33463754a92c84330d1dade396c802001e9ee4f4 doc_id: 791726 cord_uid: 3l3ah3o6 nan is another serious complication. About one-third of deaths have been attributed to thrombotic events, 4 and thrombotic complications frequently have been observed among patients hospitalized with COVID-19, even in those under anticoagulation. 3, 4 Congenital bleeding disorders (CBDs) are accompanied by various underlying disorders, including cardiovascular diseases, potentially making them more susceptible to the severe form of COVID-19. 5 The effect of a disease with a hypercoagulable state on patients with inherited hypocoagulability is illuminating. Only a few case reports and small case series are available in this context. 6, 7 Our primary observation revealed a more favorable outcome of COVID-19 on patients with CBDs. 6 We found a protective effect on patients with CBDs against COVID-19 hypercoagulability and therefore a lower rate of morbidity and mortality. In the present study, we prospectively assessed the patients with CBDs infected by SARS-CoV-2 infection. This prospective study was conducted on patients with CBDs from June 2020 to September 2020. The study was approved by the ethical committee of Shahid Beheshti University of Medical Sciences, and written consent was obtained from all participants. All patients with CBDs who were referred to the Iranian Comprehensive Hemophilia Care Center (ICHCC) for their routine check-up, prophylaxis, or on-demand therapy were included in the study. All patients were checked for body temperature, and oxygen saturation (SpO2) using pulse oximetry and were interviewed about signs and symptoms of COVID-19. All patients were checked by complete blood count (CBC) (Sysmex kx_21 haematology analyser), C-reactive pro- Iranian patients with CBDs since the pandemic's onset, appearing to show a potential protective effect against COVID-19 on patients with CBDs. 6 In an earlier study, we reported a patient with type 1 VWD and a thrombotic event 6 ; this did not occur in the study at hand. Since thrombotic events are among the leading causes of death in patients with COVID-19, absence of thrombosis significantly decreases the rate of mortality, as observed in this study. The majority of patients have had a mild COVID-19 related phenotype, as has been reported elsewhere. 9 Our hypothesis was that CBD patients with moderate-to-severe deficiency are protected against COVID-19-related hypercoagulability. This study showed that the rate of COVID-19 is not low among patients with CBDs, who probably are enjoying a favorable outcome. A considerable number of patients with CBDs and COVID-19 are asymptomatic, as in the general population. The international society of thrombosis and hemostasis (ISTH) recommended anticoagulation for all hospitalized patients to forestall thrombotic morbidity or mortality. 10 Our data demonstrated that hypocoagulability plays a major role in decreasing the rate of morbidity and mortality in patients with COVID-19, emphasizing the role of anticoagulation therapy, despite controversial reports on the effectiveness of anticoagulation. 11, 12 Although some types of CBDs have a propensity to thrombotic events, most present a state of general hypocoagulability, making patients less prone to thrombotic events, even in COVID-19, which has a tendency to a state of hypercoagulability. 6 In patients with VWD, particularly type III, pulmonary thrombosis-proposed as the main pulmonary complication in COVID-19-really is less probable. Moreover, a venous thromboembolism (VTE), particularly deep vein thrombosis (DVT), as a common complication of COVID-19, is less likely in patients whose severe factor deficiency interrupts the coagulation cascade. 4 Our other study also revealed that even COVID-19 can increase acute phase reactants, including coagulation factors, lessening the severity of the bleeding tendency, a phenomenon that could be named a "reciprocal effect." During the study period, the seven patients who were under regular prophylaxis did not experience a more serious course of COVID- 19. It appears that replacement therapy does not eliminate the CBD-related protective effect. A replacement therapy that raises the deficient factor level to normal range might eliminate this protective effect. A more flexible replacement therapy could be considered. Although data of the present study are valuable, further studies on a large number of patients are require to answer all questions about the effects of COVID-19 on the life of patients with CBDs. congenital bleeding disorders, COVID-19, hypercoagulability, hypocoagulability, SARS-CoV-2 We highly appreciate Daisy Morant's valuable work in improving the English language of the manuscript. The study was approved by Shahid Beheshti University of Medical Sciences. The authors have no competing interests. A. Dorgalaleh designed the work and wrote the manuscript. Sh. Data are available upon request. (JHU). 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Available at www.arcgis.com/apps/opsda shboa rd/index.html#/bda75 94740 fd402 99423 467b4 8e9ecf6 Registry of patients with congenital bleeding and covid-19 in Madrid ISTH interim guidance on recognition and management of coagulopathy in COVID-19: a comment Anticoagulant treatment is associated with decreased mortality in severe coronavirus disease 2019 patients with coagulopathy High incidence of venous thromboembolic events in anticoagulated severe COVID-19 patients