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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.

Background: Hemophilia B (HB), an X-linked recessive inherited bleeding disorder, exhibits a high prevalence among males. Objectives: To present the first national cohort of persons with HB to define the demographics, clinical characteristics, and treatment patterns in Turkey. Methods: This multicenter, retrospective study included 433 alive persons with HB registered in 35 centers between 1961 and 2018. Analyses were performed by age subgroups (0-17 years, 18-64 years, and ≥65 years), disease severity by factor levels (severe, 5 IU/dL). Additionally, patients were stratified based on the initiation year of follow-up at the relevant study center, creating 2 periods: 1993-2006 (referred to as period A) and 2007-2018 (referred to as period B). Results: Predominantly male (98.6%), the median age at data entry was 22.1 years (n = 429). The majority (49.0%) had moderate HB, followed by severe (30.0%) and mild (15.7%) disease. Of the 377 patients with complete treatment details, 209 (55.4%) were under prophylaxis from their diagnosis onwards, while 79 patients (21.0%) only received on-demand treatment. Additionally, 89 patients (23.6%) initially underwent on-demand treatment and later were switched to prophylaxis. Knees were the primary site of bleeding and the most frequently intervened joints. Most of the major (47.5%) and minor (53.3%) orthopedic procedures were carried out in persons with severe HB, while half of radioactive synovectomy procedures were performed on persons with moderate HB. Conclusion: This paper describes the demographics, clinical characteristics, and treatments patterns of a large cohort of alive persons with HB on a national scale.

Introduction and Aim: Propranolol is an effective treatment option for infantile hemangiomas, but there is still insufficient information about neurodevelopmental side effects of propranolol. In our study, the neurodevelopmental levels of infantile hemangioma patients receiving propranolol treatment were examined using the Bayley-III test. Method: In our single-center, cross-sectional study, patients were recruited between 1 January 2020 and 31 December 2023. In total, 40 children (n1) diagnosed with hemangioma who received propranolol treatment and 31 children (n2) who were only under observation were included. A control group of 31 healthy children (n3) matched for age and gender was also included. The demographic, clinical, perinatal, and postnatal characteristics of the total 102 children were recorded from their medical records. Neurodevelopmental levels were assessed with the Bayley-III test. The significance level was set at (p < 0.05). Results: The Bayley-III test composite and percentile scores were used to evaluate the neurodevelopmental levels. Significant differences in motor functions were found between the treated and untreated groups compared to the healthy control group (p = 0.006 and p = 0.006). However, no significant differences were found in cognitive, language, and social-emotional skills. Cognitive, language, and motor functions were associated with maternal education level, and additionally, cognitive functions were also associated with paternal education level. Conclusions: Propranolol has a relatively safer side effect profile, and therefore, it has been described as a safe agent. In our study, no significant effect of propranolol on neurodevelopment was observed. The difference in motor skills shown was mainly between the healthy control group (n3) and the treated and untreated group (n1 and n2), which led to the conclusion that the relevant difference could be due to factors other than propranolol itself.

Case report: The 22-month-old male and 15-day-old female patients presented with persistent stridor since birth. Tracheoscopy of the first patient revealed a 90% obstructing hemangioma in the subglottic area, while the second patient's CT scan showed a hemangioma at the subglottic level. Both patients were initiated on propranolol therapy. These cases highlight the significance of subglottic hemangioma as a treatable cause of stridor in infants and emphasize the importance of propranolol treatment.

Introduction: Neurofibromatosis type 1 (NF-1) is the most prevalent autosomal dominant genetic disorder. NF-1 vasculopathy is a significant complication of the disease. It affects both arterial and venous blood vessels of all sizes. Also Moyamoya syndrome is a cerebral vasculopathy . It is rarely detected with NF-1 in the pediatric group. Herein, we report of a 5 year-old male with NF1 and moyamoya syndrome. Case Report: A 6-month-old baby boy was brought by his family with the complaint of brown spots on the body. On physical examination, cafe au lait spots on the body, subcutaneous nodule in the occipital area and hypotonicity were found. He was examined considering neurofibromatosis, one of the neurocutaneous diseases. Abdominal ultrasonography, brain MRI, echocardiyography, electroencephalography were normal. There was no pathological evidence in eye examination. In genetic tests, NF1 p.GIn2217 gene was found heterozygous positive. patient was followed up with annual brain MRI. Hamartomatous lesions in left putamen and left thalamus posterior were detected in brain MRI when the patient was two years old. There was no pathological evidence on norological physical excamination.When he was three years old thickening and enhancement of the right optic nerve was found in MRI due to possible optic glioma. Only in the left cerebellar hemisphere, two millimeter-sized hamartomatous lesions in the white matter were found to have newly developed. Both internal carotid arteries (ICA) are thinned from the supraclinoid segment. Middle cerebral artery (MCA) M1 segment on the right and anterior cerebral artery (ACA) A1 segment on the left could not be selected. Many thin collateral vascular structures were selected in these localizations and were found to be significant in terms of Moyamoya disease.Regression was detected in right optic glioma.No predisposing factor was found in the examinations of the patient for thrombosis. Acetyl salicylic acid prophylaxis was started. The patient was taken under neurosurgery follow-up for revascularization surgery. The follow-up and treatment continues. Conclusion: Association of NF-1 and Moyamoya syndrome is rare, but carries a potential risk of clinicoradiological progression. Closed monitoring of children with neurofibromatosis type 1 enables early diagnosis of moyamoya syndrome. Revascularization surgery may effective way to prevent progression of clinical symptoms, but long-term results require close follow-up studies.

Objective: It is known that there were transportation problems to the hospital and treatment experienced in many disease groups during the pandemic process. The negative impact of the pandemic is particularly evident in chronic diseases and in situations that require continuous treatment. In this study, data on access to treatment and disease status in patients with bleeding diathesis were collected by questionnaire method, and the effects of the pandemic on these patients were determined. Methodology: Fifty patients who were followed up in Istanbul Medical Faculty Pediatric Hematology-Oncology Department between 2010-2022 with the diagnosis of bleeding diathesis and accepted to participate in the survey were included in the study. Questions were answered by telephone. Responses were analyzed using SPSS. Results: The mean age of the patients in our study was 13 years, the age range was between 2-26 years. The median age was 13. Of these patients, 44 (88%) were male and 6 (12%) were female. 88% of the patients were diagnosed with Hemophilia A, 12% with Hemophilia B. While 56% of the patients were receiving prophylaxis for the treatment of hemophilia, 44% were receiving treatment in case of bleeding. Sixtyfour percent of the patients went to a health institution or doctor once every 1-3 months, 18% every 6 months, 6% once a year for control and follow-up purposes. The last drug or dose change was made 0-6 months ago in 16% of the patients, 7-12 months ago in 4%, and 22% 1-2 years ago. However, in 6%, more than 2 years had passed since the last change, and 42% did not change. Serious psychiatric problems were observed in our two patients. Fear of death and anxiety disorder has been seen in a 10-year-old patient. During this period, severe hyperactivity developed in 1 patient.While 10% of the patients interrupted their treatment in the last 3-4 months, 90% did not. The reason for the disruption of the patients who interrupt their treatment is Covid infection in 20% and the drug cannot be obtained in 40%. While 94% of the patients had no problem in the supply of the drug due to the Covit-19 pandemic, 6% had a problem in the supply of the drug.While 33% of the patients who had problems in the supply of the drug received support from their doctor, 33% from the patient association to solve the problem, 33% did not receive any support from anyone. Among the reasons for having problems in the administration of the drug, 33% of the patients did not go to the hospital because they were afraid of the pandemic, 33% of them could not get treatment even though they went to the hospital, and 33% of them other reasons were reported.While 48% of the patients want an experienced health personnel to go to their home to perform their treatment, 52% do not want it, stating that they do not need it. None of the patients whose treatment was interrupted did not complain of bleeding during this period. Conclusion: It was seen that the patients experienced disruptions related to access to medication and treatment during the pandemic process. However, there were no major problems in this process, thanks to the help of their physicians and other institutions. It is important to emphasize the importance of treatment in hemophilia patients and to have easy communication with the center followed in order to overcome the pandemic process without complications.

Objective: Immune thrombocytopenia (ITP) is a rare autoimmune disease and hematologic disorder characterized by reduced platelet counts that can result in significant symptoms, such as bleeding, bruising, epistaxis, or petechiae. The thrombopoietin receptor agonist eltrombopag (EPAG) is a second-line agent used to treat chronic ITP purpura in adults and children. Materials and Methods: The present retrospective study evaluated the efficacy, safety, and side effects of EPAG treatment in pediatric patients with acute refractory and chronic immune thrombocytopenia, particularly focusing on iron-deficiency anemia. Results: The diagnosis was chronic ITP in 89 patients and acute refractory ITP in 16 patients. The mean age of patients was 9.5+-4.5 years (minimum-maximum: 1.2-18 years) at the beginning of EPAG treatment. The overall response rate was 74.3% (n=78). The mean time for platelet count of ≥50x109/L was 11.6+-8 weeks (range: 1-34 weeks). The treatment was stopped for 27 patients (25.7%) at an average of 6.8+-9 months (range: 1-38 months). The reason for discontinuation was lack of response in 18 patients, nonadherence in 4 patients, and hepatotoxicity in 2 patients. Response to treatment continued for an average of 4 months after cessation of EPAG in 3 patients. Conclusion: Results of the current study imply that EPAG is an effective therapeutic option in pediatric patients with acute refractory and chronic ITP. However, patients must be closely monitored for response and side effects during treatment, and especially for iron deficiency.

Objective: PTEN/AKT pathway deregulations have been reported to be associated with treatment response in acute leukemia. This study examined pediatric T-cell acute lymphoblastic leukemia (T-ALL) samples for PTEN and AKT1 gene variations and evaluated the clinical findings. Materials and Methods: Fifty diagnostic bone marrow samples of childhood T-ALL cases were investigated for the hotspot regions of the PTEN and AKT1 genes by targeted next-generation sequencing. Results: A total of five PTEN variations were found in three of the 50 T-ALL cases (6%). Three of the PTEN variations were first reported in this study. Furthermore, one patient clearly had two different mutant clones for PTEN. Two intronic single-nucleotide variations were found in AKT1 and none of the patients carried pathogenic AKT1 variations. Conclusion: Targeted deep sequencing allowed us to detect both lowlevel variations and clonal diversity. Low-level PTEN/AKT1 variation frequency makes it harder to investigate the clinical associations of the variants. On the other hand, characterization of the PTEN/ AKT signaling members is important for improving case-specific therapeutic strategies.

Objective: A questionnaire form was prepared by the Turkish Pediatric Hematology Society- Subcommittee of Hemostasis, Thrombosis and Hemophilia to determine the current approaches in the diagnosis and treatment of childhood ITP in our country. Our aim was to share the results of this study, and to do new, national, multicenter prospective studies. Methodology: This form, which consists of twenty questions with multiple choices, but a brief explanation is requested when there is a different approach other than the options given, was sent to all pediatric hematologists via e-mail. Results: The response was obtained from 55 hematologists experienced in ITP from 47 centers in total. Due to space constraints, this summary could not present the survey questions and answers. Conclusion: In conclusion, the approaches for diagnosis and management of childhood ITP differ between centers.

Objective: Chronic myeloid leukemia (CML) is rarely seen in children. The development of myelofibrosis in CML is not uncommon and is associated with a poor prognosis. In cases unresponsive to treatment, tyrosine kinase mutation should be checked for drug resistance, second generation tyrosine kinase inhibitor (TKI) drugs (dasatinib/nilotinib) should be switched to and a suitable donor for bone marrow transplantation should be sought. Third-choice TKI can be used in children who are unresponsive to treatment and do not have a suitable donor. Materials and Methods: Experience of third-choice TKI(ponatinib) in a child with CML diagnosis due to unresponsiveness to treatment. Results: A 5.5-year-old female patient with no known disease was referred to us because of hepatosplenomegaly (liver 5 cm, spleen 10 cm). There was no laboratory disorder except for anemia (hgb 8.9 g/dL) and high LDH (1104 U/L). WBC was 11.1 × 103/µL neu 6.92 × 103/ µL plt 304000/µL. Peripheral smear showed leukoerythroblastosis. Bone marrow biopsy result was evaluated as compatible with myelofibrosis and an increase in blast rate from 8% to 18% in the bone marrow. The patient was diagnosed CML accelerated phase with cytogenetic (46,XX,t(9:22)(q34;q11))and translocation (t(9:22)- p210,BCR/ABL positive) results and. Imatinib treatment was started at 400 mg/m2.The copy number of BCR-ABL p210 checked before treatment was 72% IS. However, the patient developed febrile neutropenia, and imatinib dose reduction (< 200 mg/m2) and interruption were required in the follow-up.Under imatinib treatment, BCR-ABL copy number was 16%IS at 1 month, 11%IS at 3 months, and 95%IS at 5 months. Due to the increase in the BCR-ABL copy number, nilotinib was switched to as a second-choice TKI(230 g/m2/dose, in 2 doses).No mutation could be detected in the c-ABL gene, which was examined for tyrosine kinase resistance. HLA groups were sent from the family and compatible donors were not found. Due to severe neutropenia in the follow-up, nilotinib could be continued at 50% dose. Under nilotinib treatment, the BCR-ABL copy number was 13% IS at 1 month, 10% IS at 2 months, and 31% IS at 3 months. The patient was started on ponatinib (18 mg/m2/day) as a third choice TKI. However, due to the deep neutropenia of the patient, it was possible to continue with a dose of 10 mg/m2 from the 2nd week. With this dose, the neutrophil is around 0.8-1 × 103/µL. Under ponatinib treatment, BCR-ABL copy number was 6.6% IS at 1 month, 0.8% IS at 3 months, 0.09% at 5 months, and 0.05% at 6 months. No significant side effects were observed except neutropenia. Conclusion: There is no approved treatment in pediatric CML cases where the second choice TKI fails and there is no donor for transplantation. FDA approval for ponatinib in adult patients was obtained in December 2020. Ponatinib is a natural or mutant pan-BCR-ABL mutation inhibitor. It also inhibits VEGFR, FGFR,PDGFR,EPH and SRC kinases as well as KIT,RET,TIE2 and FLT3. The use of ponatinib should be evaluated by monitoring side effects/tolerance in pediatric cases where there is no other treatment option, and there is a need for studies on this subject.

Objective: To investigate the clinical impact of vancomycinresistant enterococci (VRE) colonization in patients with hematologic malignancies and associated risk factors. Materials and Methods: Patients colonized and infected with VRE were identified from an institutional surveillance database between January 2010 and December 2013. A retrospective case-control study was performed to identify the risk factors associated with development of VRE infection in VRE-colonized patients. Results: Fecal VRE colonization was documented in 72 of 229 children (31.4%). Seven VRE-colonized patients developed subsequent systemic VRE infection (9.7%). Types of VRE infections included bacteremia (n=5), urinary tract infection (n=1), and meningitis (n=1). Enterococcus faecium was isolated in all VRE infections. Multivariate analysis revealed severe neutropenia and previous bacteremia with another pathogen as independent risk factors for VRE infection development in colonized patients [odds ratio (OR): 35.4, confidence interval (CI): 1.7-72.3, p=0.02 and OR: 20.6, CI: 1.3-48.6, p=0.03, respectively]. No deaths attributable to VRE occurred. Conclusion: VRE colonization has important consequences in pediatric cancer patients.

Objective: Mild bleeding symptoms are commonly seen in the general population. The aim of this study was to determine the final clinical and laboratory features of children referred for a first evaluation with a suspected bleeding disorder in the pediatric outpatient clinic of İstanbul University. Materials and Methods: The medical records of 26,737 outpatients who were admitted to the Division of Ambulatory Pediatrics between 31 October 2011 and 31 October 2012 were evaluated retrospectively. Ninety-nine patients were initially diagnosed as having probable bleeding disorders and were followed up. The symptoms of bleeding in addition to coagulation tests were analyzed. Results: Of the 99 patients, 52 (52.5%) were male and 47 were female, and the mean age of the entire study group was 9.1+-4.1 years (minimum-maximum: 2-18 years). Major bleeding symptoms were epistaxis in 36 patients (36.4%), easy bruising in 32 (32.3%), and menorrhagia in 6 (6.1%). After initial tests ordered by the pediatrician, 36 of 99 patients (36.4%) were diagnosed as having bleeding disorders that included von Willebrand disease in 12 (12.1%), hemophilia A or B in 9 (9.1%), and other rare factor deficiencies in 9 (9.1%). Six patients (6.1%) were found to have combined deficiencies. Seven of 36 patients had a family history of bleeding. Conclusion: Among the patients referred for bleeding disorders, 36.4% were diagnosed with a bleeding disorder with the help of primary screening tests ordered in the outpatient clinic.

OBJECTIVE: To identify the well-known common translocations and FLT3 mutations in childhood acute myelogenous leukemia (AML) patients in Turkey. 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, our case shows a better survival and this might be due to the complementation effect of the t(15;17) translocation. The lower mutation rate (4%) of FLT3 gene seems to be one of the first results for Turkish population.

OBJECTIVE: We evaluated the clinical findings, laboratory data, management, and outcome of children with rare coagulation deficiencies (RCDs) diagnosed in Turkey during the past 10 years, retrospectively. METHODS: A simple Excel file of questionnaire was sent to contact people in each center to standardize data collection by Turkish Society of Pediatric Hematology, Hemophilia-Thrombosis-Hemostasis subcommittee. RESULTS: Total of 156 patients were included in this study from 12 pediatric referral centers. Most common rare coagulation disorders in the study was as follows: FVII (n: 53, 34%), FV (n: 24, 15.4%), FX (n: 23 case, 14.7%). The most common initial findings in our patients were epistaxis, echymosis and gingival bleeding in all type of RCDs respectively. CONCLUSION: First symptoms are mucosal bleedings and FFP and tranexamic acid is still the most commonly used therapeutic options. We suggest that prophylactic treatment that we use for hemophilia patients should be considered at the very beginning among the therapeutic options for rare factor deficiencies with severe clinical course and factor deficiency that can lead to severe bleeding.

Objective: Respiratory viruses are an important cause of morbidity and mortality in pediatric hematology oncology patients. We aimed to determine the infection rate, clinical and epidemiological characteristics of respiratory viruses in pediatric patients with hemato-oncological malignancy, aplastic anemia and congenital neutropenia and to show how these viruses affect the primary disease course and treatment. Methodology: Between August 2015 and December 2018, 97 patients aged between 5 months and 215 months who were admitted to Istanbul University, Istanbul Faculty of Medicine, Department of Pediatric Haematology-Oncology with acute respiratory tract infection findings and diagnosed with Haemato-Oncological Malignancy, Congenital Neutropenia, Aplastic Anaemia and who had viral respiratory panel were retrospectively analysed. In the viral respiratory panel test, nasal swab samples of the patients were evaluated by RT-multiplex PCR method. SPSS (Statistical Package for the Social Sciences) 22.0 programme was used for statistical analyses Results: A total of 97 patients, 52 males (53.6%) and 45 females (46.4%), aged between 5 months and 215 months (78.81±60.17 months, median 60 months) were included in the study. The most common viral respiratoty panel (VRP) positivity was observed between 5 months and 208 months and the mean age was 85.49±61.73 months (median=81 months). Although 44.3% (n=43) of the patients presented in winter and 23.7% (n=23) in autumn, VRP positivity was more common in patients presenting in spring (n=43, 70%) and winter (n=22, 51.2%) seasons. When the VRP results of the patients were analysed; 50.5% (n=49) were positive; 39.2% (n=38) were monoinfection, 11.3% (n=11) were co-infection) and 49.5% (n=48) were negative. When we looked at the VRP results, rhinovirus (hRV) was the most common virus with a frequency of 22.4% (n=11). Other viruses were Respiratory Synsititial Virus (RSV) A/B (14.2% n=7), Parainfluenza (14.2% n=7), Influenza (8.2% n=4), Coronavirus (8.2% n=4), Metapneumovirus (2.1% n=1), Mycoplasma pneumonia (6.1% n=3). Among the co-infections seen in a total of 11 patients, hRV and RSV A/B were the most common viruses accompanying other viruses with a rate of 63.6% (n=7). Among a total of 67 patients who were in various stages of CT and whose treatment was completed, the most common VRP positivity was seen in patients in the induction phase with a rate of 28.3% (n=19). Of the 12 patients with co-infection, 5 (41.6%) were in the induction phase. Cough (n=59 60.8%) and fever (n=47 48.5%) were the most common presenting complaints, accompanied by wheezing (n=17 17 17.5%), respiratory distress (n=11 11.3%), diarrhoea/vomiting (n=9 9.3%) and muscle pain (n=9 9.3%). VRP was positive in 43.9% of patients presenting with fever. The most common hRV virus was found most frequently in spring and winter seasons. Viral respiratory infection positivity was most frequently seen in ALL (n=16 33.3%), second most frequently in Hodgkin's Lymphoma (n=5 10.5%) and Neuroblastoma (n=5 10.5%). Among the patients, upper respiratory tract infection (URTI) (74.2%, n=72) was more common than lower respiratory tract infection (LRTI) (25.8%, n=25). The rate of LRTI in co-infections (28.0%, n=14) was higher than the rate of URTI (6.9%, n=5) and was statistically significant (p=0.021). When hemogram and biochemistry results were analysed, although neutropenia (50.5%) and lymphopenia (50.5%) were observed at a high rate in patients with positive VRI, they were not statistically significant when compared with VRP positivity. Of the patients with VRP positivity (50.5% n=49), 34.6% (n=17) required hospitalisation due to viral respiratory infection. Of the patients included in the study, 4 patients need intensive care unit due to bacterial pneumonia (Mycoplasma pneumonia and Pneumocystis jireovici), bleeding into a mass (hepatoblastoma) and pericardial effusion (peripheric T cell lymphoma). In 7 patients whose chemotherapy duration was prolonged, the duration of treatment prolongation ranged between 4 and 60 days (mean 19.29±20.69 and median 10 days). No VRI-related mortality was observed among the patients during the follow-up period. Conclusion: Identification of respiratory viruses in pediatric hematology oncology patients contributes to the management of their primary disease.

Objective: The aim of our study is to determine demographic data in patients with thrombosis in childhood to determine hereditary and/or acquired risk factors that cause thrombosis, to diagnose and treat thrombosis, to detect the complications related to thrombosis or treatment, to examine mortality and morbidity after thrombosis, and to evaluate the final status of the patients. Methodology: 160 cases diagnosed with thrombosis between the ages of 1 month and 18 years, who were followed up by the Pediatric Hematology and Oncology outpatient clinic of IstanbulSchool of Medicine, between 01-JAN-2012 and 01-JAN-2022 were analyzed, retrospectively. While obtaining the medical data of the patients, patient files and hospital information management systems were used. The obtained data were analyzed with IBM SPSS V23 computer program and p

Objective: The bleeding phenotype of patients with inherited FVII deficiency is variable, and epistaxis is one of the most frequent symptoms. Interestingly, the bleeding risk does not correlate with the level of FVII activity. The severity of FVII deficiency and the type of surgery are not determinants of the optimal management of surgery, the doses and the duration of rFVIIa therapy are widely variable. The aim of this study is to present our successful experience in a 16-year-old boy with inherited FVII deficiency and a huge nasopharyngeal angiofibroma with a very high risk of bleeding Case report: The patient was referred with recurrent epistaxis in the last 6 months and he was diagnosed as an inherited FVII deficiency (FVIIC:29%, FVII inhibitor negative with positive family history). Tranexamic acid (10days) and rFVIIa (2doses) were used with success in the management of this surgery. Since this surgery may cause life-threatening bleeding, endovascular particle embolization was done to the important vessels feeding the mass one day before surgery without rFVIIa support. No bleeding or thrombosis were observed in our patient. Conclusion: In conclusion, a life-threatening major surgery was successfully done for a patient with inherited FVII deficiency and a huge angiofibroma. However, perioperative management of patients with FVII deficiency still remains a major challenge and clinical trials are needed to provide evidence-based optimal management of surgeries. And, angiofibroma should be thought in the differential diagnosis of epistaxis.

Objective: HLA class I and II alleles are shown to be associated with certain diseases. A restricted numbers of alleles were found to be related to alloimmunisation in thalassemia population. The role of human leucocyte antigens in thalassemia is trend topic. In this study, the aim was to evaluate the differences in HLA frequencies of beta thalassemia patients comparing with healthy controls. Methodology: The data were collected of 100 patients who were diagnosed with beta thalassemia and 100 healthy controls were included in the study. The low resolution HLA-A, -B, -DRB1, tissue group data were performed Istanbul University, Faculty of Medicine, Medical Biology Department HLA typing laboratory. All data were analyzed retrospectively and their HLA allele frequencies were analyzed by SPSS (v22) program. Results: We found an increased frequency of HLA-B*14 (8% versus 2%) and HLA-B*52 (17% versus 2%) compared to the control group (p=0.05, OR=4.26; p