COVID-19 in patients with congenital bleeding disorders in Russia

Abstract

Background. Internationally published data about the course of COVID-19 in patients with congenital bleeding disorders (CBDs) are limited. There are questions about how COVID-19 affects the course of CBDs and, conversely, how CBDs affect the course of coronavirus infection.Aim — to analyze the course of COVID-19 in patients with CBDs in Russia.Materials and methods. A cross-sectional survey was conducted at the National Medical Research Center for Hematology (Moscow) for the period from June 25 to July 31, 2022. A cluster of 187 patients from different regions and cities of Russia were interviewed with a questionnaire based on survey administration software (Google forms) containing 27 questions.Results. COVID-19 affected 115 (62 %) of 187 surveyed patients and 22 (19 %) patients suffered from coronavirus infection twice. Hospitalization was required for 14 (12 %) patients with an average age of 42 years (10 patients with severe hemophilia A, 1 patient with moderate hemophilia B, 2 patients with von Willebrand disease and 1 patient with hypoproconvertinemia). During COVID-19 bleeding was observed in 9 (8 %) patients and was represented by hemarthrosis, ecchymosis, hematomas, nosebleeds, menorrhagia, hemorrhoidal bleeding, hemorrhage in the eyeball. There were no cases of thrombosis among 115 COVID-19 patients. Among patients with CBDs who had COVID-19, compared with patients who had not COVID-19, von Willebrand disease was statistically significantly more common (p = 0.04). Changes in the course of CBD after COVID-19 were noted by 21 (18 %) of 115 patients: 11 (10 %) of those who were ill noted increased joint pain, 9 (8 %) complained of joint pain that had not been previously experienced; 10 % of patients described changes of hemorrhagic syndrome.Conclusion. The probability of hospitalization of patients with CBDs and COVID-19 older than 40 is statistically significantly higher. Von Willebrand disease can be considered as a potential risk factor for COVID-19. Given the absence of cases of thrombosis in the interviewed group of patients, the results of the study suggest that the presence of hypocoagulation in patients with CBDs may be a protective pathophysiological mechanism that prevents the development of COVID-19-associated thrombotic complications.

Congenital Bleeding Disorders and COVID-19—A Systematic Literature Review

“…However, information about the severity of hemophilia A was not available in seven studies ( n = 4,232; 93.7%). 14 15 16 18 19 20 21 Out of 4,517 patients with hemophilia A, an inhibitor was observed in 29 patients (0.6%) across four studies. 7 8 12 14 However, information about inhibitors in hemophilia were not provided in eight studies ( n = 4,029; ∼89%).…”

“…12 In addition, it was reported that two patients (<1%) had factor XIII (FXIII) deficiency, 7 13 while five patients (<1%) each were reported to have FVII deficiency and FX deficiency. 12 18 20 21 FV deficiency was reported in two patients (<1%), and congenital fibrinogen disorder and FXI deficiency were reported in one patient each (<1%). 13 20 Three studies did not differentiate between the types of RBD ( n = 13; 0.1%).…”

“…7 8 12 14 However, information about inhibitors in hemophilia were not provided in eight studies ( n = 4,029; ∼89%). 13 15 16 17 18 19 20 21 Of the 877 patients with hemophilia B, an inhibitor was reported in seven patients (0.8%) across two studies. 8 14 The number of patients with severe, moderate, and mild hemophilia B was 28 (3.2%), 8 (∼1%), and 9 (∼1%), respectively.…”

“…However, information about the severity of hemophilia B was not available in six studies ( n = 832; 94.8%). 14 15 16 18 19 21 Furthermore, in one study, 19 cases (0.2%) were classified as unspecified hemophilia, as they had a single diagnosis code for both hemophilia A and B in the database. 14 Furthermore, hemophilia carriers ( n = 31; 0.3%) were reported in two studies.…”

“…The exact number of patients with hemophilia (A or B) and COVID-19 was not reported in four studies ( n = 215, 29.2%) ( Tables 1 and 2 ). 8 15 17 21 Out of 352 patients with hemophilia A and COVID-19, 55 (15.6%), 2 (0.5%), and 13 (3.9%) had severe, moderate, and mild hemophilia A, respectively. Information regarding the severity of hemophilia A in the remaining patients ( n = 282; ∼80%) was not reported.…”