Clinical and Laboratory Evaluation of Adult Hemophilia A and Hemophilia B Patients Single Center Experience

Aim: Hemophilia is an X-linked congenital bleeding disorder caused by deficiency of coagulation factors factor VIII (hemophilia A) or factor IX (hemophilia B). Our aim in this study was to create our own data by checking the clinical classifications of hemophilia patients, the frequency of inhibitory development and whether they had joint bleeding clinically, and to compare these data with the literature. Methods: 64 male patients who were diagnosed with hemophilia and applied to the Internal Diseases Adult Hematology Outpatient Clinic of Dicle University Medical Faculty Research and Practice Hospital between December 2010 and April 2018 were included in this study. Inhibitor levels, complete blood count, biochemistry, some hormone and vitamin parameters were evaluated by looking at the laboratory results of our patients. Any joint bleeding or signs of intracranial bleeding were investigated on radiological imaging. Results: A total of 64 patients were included in our study.The mean age of our patients was calculated as 26.4 ± 8.9 years (17-60 years). Of the patients, 85.9% (n = 55) were grouped as hemophilia A, and the remaining 14.1% (n = 9) as hemophilia B. Inhibitor level was found to be negative in 85.9% (n = 55) of our patients. The rate of our patients who were considered to have developed inhibitors was 14.1% (n = 9). In 47.6% of the patients (n = 30), joint bleeding was detected in radiological imaging, and in the remaining 52.4% (n = 33) there was no bleeding in any joint. Conclusion: Accessing the demographic data of these patients in the rare disease group and requiring special follow-up, what problems await the patients during the follow-up period and in this study, in which we aimed to understand the needs of these patients, it is very important that our patients have more information about the joint bleeding that affects the quality of life of the patients and ages with the patient, and it is very important to increase the number of responsibility and awareness projects that will integrate them into social life and we think that we need more literature studies on this subject.