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Article: Korte, W., Poon, M., Iorio, A. et al. (1 more author) (2017) Thrombosis in inherited fibrinogen disorders. Transfusion Medicine and Hemotherapy, 44 (2). pp. 70-76. ISSN 1660-3796 https://doi.org/10.1159/000452864

This is the peer-reviewed but unedited manuscript version of the following article: Transfus Med Hemother 2017;44:70-76 (DOI: 10.1159/000452864). The final, published version is available at http://www.karger.com/?doi=10.1159/000452864.

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[email protected] https://eprints.whiterose.ac.uk/ Table 1. Clinical studies and case series of a- hypo- and dysfibrinogenemia patients where thrombosis was reported

Authors and year of Country Study population Methods Thrombotic events publication

Lak et al. 1999 [87] Iran 55 patients (28 F 27 M) with Retrospective study of symptoms in 2 patients (4%) with afibrinogenemia developed afibrinogenemia. Age range 2 73 afibrinogenemia vs. hemophilia patients thrombotic symptoms years

Kreuz et al. 2005 [88] Germany 12 patients (8 F, 4 M) with a- Retrospective study investigating the effects One patient with afibrinogenemia developed venous hypo- or dysfibrinogenemia. of fibrinogen therapy for bleeding episodes thrombosis and non-fatal lung embolism during the Median age: 11.5, range 1 day to treatment. May have been related to fibrinogen 29 years

Santacrose et al. 2006 Italy 18 patients (10 F, 8 M) with a- Retrospective study of patients treated in the An 11 year old boy with cerebral of [89] hypo- or dysfibrinogenemia. Age clinic over a 10 year time period the left sagittal sinus was the only case presenting with range: 1 53 years thrombosis

Casini et al. 2015 [7] Switzerland 101 patients (68 F, 33 M) with Multicenter study of 101 patients with There were 28 first thrombotic events, including 20 dysfibrinogenemia. Mean age at congenital dysfibrinogenemia to characterize venous events (11 deep venous thromboses, 3 PEs, 2 clinical diagnosis: 29.2 years, the incidence of hemorrhagic and thrombotic superficial vein thrombophlebitis events, and 4 standard deviation: 16.8 years events as well as complications of pregnancy thromboses at unusual sites) and 8 arterial events (4 and surgery , 2 acute myocardial infarctions, 1 peripheral artery occlusion, and 1 mesenteric artery thrombosis. Fourteen of these events occurred at time of diagnosis, and 14 during follow-up

Miesbach et al. 2010 [90] Germany 37 patients with hereditary Laboratory and clinical evidence of 19% of probands (9/37, all above age of 50 years), had dysfibrinogenemia. 63% F, 37% hemostatic abnormalities were assessed experienced at least one episode of arterial or venous M. Age: median 58 years, range: thrombosis. Among these, were two (7%) with deep 11 86 years venous thrombosis, seven with arterial thrombosis, and five (14%) had experienced both

Table 1 continued on next page

Table 1. Continued

Authors and year of Country Study population Methods Thrombotic events publication

Nguyen et al. 1998 [91] France 12 patients (4 F, 8 M) with Retrospective analysis of patients with 4 patients with dysfibrinogenemia had various types of dysfibrinogenemia, 4 with dysfibrinogenemia presenting with or thrombotic episodes thrombosis, 8 without. Age without thrombotic episodes range: 66 24 years

Ramanathan et al. 2013 Denmark Family (N = 14) with Case description of a family affected by Deep venous/arterial thrombosis or pulmonary [92] dysfibrinogenemia dysfibrinogenemia and thrombosis embolism reported in 4 family members with (FGA ) dysfibrinogenemia

Shapiro et al. 2013 [93] UK 35 patients with heritable Historical symptoms of thrombosis were Thrombosis at any site: 3 (9%) dysfibrinogenemia. 20 F, 15 M. determined by patient interviews and Pulmonary embolus: 2 (6%) Age: median 38 years, range: 11 inspection of hospital records Thrombotic : 1 (3%) 78 years

Zhou et al. 2015 [11] China 102 patients with congenital Thrombosis at any site: 4 (3.9%) dysfibrinogenemia. 49 F, 53 M. were recorded and quantified using Lower extremity venous thrombosis: 2 (2.0%) Age: median 35 years, range: 3 consensusPatients previous ISTH bleeding clinical assessment manifestations tool : 1 (1.0%) 76 years Portal vein thrombosis: 1 (1.0%) Dorsal vein of foot thrombosis: 1 (1.0%)