Özet:

Objective: The aim of this retrospective study was to evaluate the clinical spectrum, demographic features and complications in patients with a hereditary coagulation disorder.Material and Methods: A total of 105 patients who were being followed-up with a diagnosis of hereditary coagulation factor defi ciency at the Pediatric Hematology Clinic of Dr. Sami Ulus Obstetrics and Gynecology, Children’s Health and Disease Training and Research Hospital were evaluated retrospectively in this study.Results: The most frequently observed disorders were hemophilia A (46.7%), von Willebrand Disease (vWD) (16.2%) and hemophilia B (8.6%). Rare bleeding disorders (RBD) were detected in 28.5% of the study group and the most common RBDs were fi brinogen (10.5%) and factor VII (7.6%) defi ciencies followed by FXI, FV and FX at 3.8%, 2.9% and 1.9%, respectively. The common initial symptom was hemarthrosis in hemophilia A and epistaxis in vWD. Eighteen patients (17%) were asymptomatic. The frequency of inhibitor development in hemophilia was 12.2%. The prevalence of hepatitis B and hepatitis C among all patients was 0.9%. Conclusion: Bleeding disorders are widely encountered in both hematology and pediatric clinics. Most patients with coagulation disorders present with bleeding symptoms but some patients may be asymptomatic or have minor symptoms that lead to a delay in diagnosis. Prompt diagnosis and treatment are critical in symptomatic patients. One has to keep in mind that not all factor defi ciencies lead to severe bleeding and patients with abnormal laboratory results also need to be investigated for familial coagulation disorders too

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