Browsing by Author "Unuvar, Aysegul"

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Analysis of Chromosomal Aberrations and Flt3 Gene Mutations in Childhood Acute Myelogenous Leukemia Patients
(Galenos Yayincilik, 2012) Coskunpinar, Ender; Anak, Sema; Agaoglu, Leyla; Unuvar, Aysegul; Devecioglu, Omer; Aydogan, Gonul; Ozbek, Ugur
Objective: To identify the well-known common translocations and FLT3 mutations in childhood acute myelogenous leukemia (AML) patients in Turkey. Material and Methods: The study included 50 newly diagnosed patients in which t(15;17), t(8;21), and inv(16) chromosomal translocations were identified using real-time PCR and FLT3 gene mutations were identified via direct PCR amplification PCR-RE analysis. Results: In all, t(15;17) chromosomal aberrations were observed in 4 patients (8.0%), t(8;21) chromosomal aberrations were observed in 12 patients (24.0%), inv(16) chromosomal aberrations were observed in 3 patients (6.0%), and FLT3-ITD mutations were observed in 2 patients (4.0%); FLT3-D835 point mutation heterozygosity was observed in only 1 patient (2.0%) patient. Conclusion: Despite of the known literature, a patient with FLT3-ITD and FLT3-D835 double mutation shows a better survival and this might be due to the complementation effect of the t(15;17) translocation. The reportedmutation rate in this article (4%) of FLT3 gene seems to be one of the first results for Turkish population.
The Impact of Factor Viii Concentrate on the Development of Inhibitors in Children With Severe Hemophilia A: Turkish Pup Registry Study
(Wiley-blackwell, 2014) Kavakli, Kaan; Unuvar, Aysegul; Salcioglu, Zafer; Kilinc, Yurdanur; Oner, Ahmet Faik; Ay, Yilmaz; Zulfikar, Bulent
Results of Multicenter Registry for Patients With Inherited Factor Vii Deficiency in Turkey
(Taylor & Francis Ltd, 2022) Akdeniz, Aydan; Unuvar, Aysegul; Ar, Muhlis Cem; Pekpak, Esra; Akyay, Arzu; Mehtap, Ozgur; Demir, Ahmet Muzaffer
Introduction: Inherited factor VII (FVII) deficiency (FVIID) is the most common of inherited rare bleeding disorders. Other determinants of clinical severity apart from FVII level (FVIIL) include genetic and environmental factors. We aimed to identify the cut-off FVIILs for general and severe bleedings in patients with FVIID by using an online national registry system including clinical, laboratory, and demographic characteristics of patients. Methods: Demographic, clinical, and laboratory data of patients with FVIID extracted from the national database, constituted by the Turkish Society of Hematology, were examined. Bleeding phenotypes, general characteristics, and laboratory features were assessed in terms of FVIILs. Bleeding rates and prophylaxis during special procedures/interventions were also recorded. Results: Data from 197 patients showed that 46.2% of patients had FVIIL< 10%. Most bleeds were of mucosal origin (67.7%), and severe bleeds tended to occur in younger patients (median age: 15 (IQR:6-29)). Cut-off FVIILs for all and severe bleeds were 16.5% and 7.5%, respectively. The major reason for long-term prophylaxis was observed as central nervous system bleeding (80%). Conclusion: Our data are consistent with most of the published literature in terms of cut-off FVIIL for bleeding, as well as reasons for prophylaxis, showing both an increased severity of bleeding and younger age at diagnosis with decreasing FVIIL. However, in order to offer a classification similar to that in Hemophilia A or B, data of a larger cohort with information about environmental and genetic factors are required.
Risk Factors for Neurologic Sequelae in Children and Adolescents With Hemophilia After Intracranial Hemorrhage
(Elsevier, 2024) Evim, Melike Sezgin; Unuvar, Aysegul; Albayrak, Canan; Zengin, Emine; Yilmaz, Ebru; Kaya, Zuhre; Oren, Hale
Background: Intracranial hemorrhage (ICH) is reportedly rare but has high morbidity and mortality risk in persons with hemophilia. Although the risk factors that facilitate bleeding are known, the factors affecting the sequelae are not well known. Objectives: We planned to investigate the risk factors for neurologic sequelae in children and adolescents with hemophilia suffering from ICH. Methods: An invitation was sent to pediatric hematology centers via email. Clinical and laboratory findings, neurologic sequelae, and recurrence of bleeding in persons with hemophilia who developed ICH were questioned. Results: Eighty-six patients from 21 centers were evaluated. All patients were less than 18 years of age at the time of ICH. Thirteen patients had ICH in the neonatal period, while 40 patients had a known diagnosis of hemophilia before ICH, and 33 patients were undiagnosed before ICH. Five patients died, 2 of whom died in the neonatal period. The rate of neurologic sequelae was 25 of 81 (30%). The most common neurologic sequela was epilepsy (n = 11/25), followed by hemiparesis (n = 5/25). Cerebral shift (odds ratio, 3.48) and development of ICH in the neonatal period (odds ratio, 4.67) were significant for the development of neurologic sequelae in multivariate analysis. On follow-up, recurrence of ICH occurred in 8 of 81 (10%). Conclusion: ICH in the neonatal period and cerebral shift were the two main risk factors for the development of neurologic sequelae. Neonatal departments must be alert to the signs of bleeding. It is important for healthcare professionals to overcome the barriers to primary prophylaxis and to take trauma-related precautions.