CLINICAL AND LABORATORY FEATURES IN PATIENTS WITH PARAHEMOPHILIA

Objective. To characterize the clinical manifestations and coagulation parameters in Azerbaijani patients diagnosed with parahemophilia (factor V deficiency). Participants and Methods. The study included 36 female patients with a confirmed diagnosis of parahemophilia. Data collection involved patient interviews and physical examinations, parental surveys, review of medical records, and anamnestic analysis. Blood samples were collected in the morning on an empty stomach, following standard clinical protocols. A complete blood count was performed using a hematology analyzer, and coagulation studies were conducted, including assessments of prothrombin time (PT), prothrombin index, INR, activated partial thromboplastin time (APTT), and levels of clotting factors. Results. Factor V plays a central role in the coagulation cascade, which consists of tightly regulated enzymatic reactions leading to thrombin formation and fibrin deposition at sites of vascular injury. Patient ages ranged from 6 months to 73 years, with a mean age of 18.0 ± 16.42 years. 47.2% of participants were of reproductive age. The most common clinical manifestations included abnormal uterine bleeding – 36.1%, postoperative bleeding – 8.3%, hereditary coagulopathy – 16.7%, epistaxis and gum bleeding – 11.1% (each), menorrhagia – 13.9%, hematuria – 2.8%. Laboratory findings showed prolonged PT in 63.9% of patients, prothrombin index < 60% in 55.6%, elevated INR in 55.6%, prolonged APTT in 72.2%, factor V deficiency in 22.2%, reduced factor X concentration in 30.6%, and prolonged clotting time (Li-White method) in 100% of patients. Additionally, two patients were found to have a combined deficiency of factors V and VIII. Conclusion. The most frequent clinical presentation was uterine bleeding, particularly in women of reproductive age. Significant laboratory abnormalities included prolonged PT, APTT, elevated INR, and deficiencies of factors V and X, as well as prolonged clotting time (Li-White method). The identification of combined factor V and VIII deficiency in two cases highlights the need for comprehensive coagulation screening. These findings contribute to improved diagnostic accuracy and management of patients with rare coagulation disorders.