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Background/aim: The transfusion of blood products is a life-saving clinical practice in patients with bleeding, hemoglobinopathy, and cancer. It was aimed herein to analyze the frequency and types of blood component-related acute transfusion reactions (ATRs) in pediatric patients. Materials and methods: This retrospective study was conducted at a tertiary care academic pediatric hospital. Results: During the study period, 30,811 transfusions were administered to 25,448 patients. There were 103 ATRs detected in 81 patients (0.33%; 3.34 reactions per 1000 transfusions, mean age 8.3 ± 5.98 years, 36 females and 45 males). All the reactions were observed within an average of 4 h after the transfusion began. The most common ATRs were allergic reactions (79; 76.6%) and febrile nonhemolytic transfusion reactions (12; 11.6%). All the allergic transfusion reactions occurred within the first hour after the start of the transfusion. Granulocyte concentrates were the blood component associated with the highest ATR rate (2.1%). Conclusion: Within our hospital, pediatric hematology-oncology wards and the stem cell transplantation unit had the most frequent ATR reports; therefore, when transfusions are carried out, increased attention should be given to these units. Educating health staff about the adverse effects of transfusion therapy should increase the awareness and reporting of ATRs in children. [ABSTRACT FROM AUTHOR]

Infections are a significant cause of morbidity and mortality of chemotherapy-induced neutropenia in children with acute leukemia. The aim of this study was to evaluate microbiologically documented infections (MDIs) during febrile neutropenia (FN) episodes and their relation to mortality. Four hundred eighty-seven FN episodes of 140 children were enrolled in this single-center study, and MDI in those FN episodes was retrospectively examined. Eighty-four patients (60%) had at least one positive peripheral blood, central line, or urine culture. MDIs were detected in 163 of 487 (33.4%) FN episodes of 84 children with leukemia. Gram-negative bacteria, Gram-positive bacteria, and fungal agents were isolated in 52.7%, 40.4%, and 6.9% of whole episodes. Coagulase-negative Staphylococci and Enterococci were the most detected Gram-positive bacteria. Klebsiella spp. and Escherichia coli were the most common Gram-negative bacteria isolated in the entire cohort. A central line was present in 145 MDI episodes, and catheter removal was required in 35 cases (17.7%) due to infection with Gram-negative bacteria, Gram-positive bacteria 43%, 28.5%, and fungus 28.5%, respectively. MDI-related mortality was 9.8%. The highest mortality rate (16.7%) was observed in Gram-negative bacteria and patients with relapsed and resistant leukemia. The most common infectious agent related to mortality was Klebsiella (31%). Resistance to third-or fourth-generation cephalosporins in Gram-negative bacteria was found to be over 50% of our cohort uri. Empirical antibiotic therapy at the onset of FN in neutropenic patients is crucial; therefore, the institution's predominant pathogens and resistance patterns should guide the choice of empirical antimicrobials. To reduce mortality and morbidity, each center should know its local epidemiological data and antibiotic susceptibility. [ABSTRACT FROM AUTHOR]

Objective: Patients with inherited bleeding disorders faced problems in accessing healthcare during coronavirus disease 2019 pandemic. This study aimed to investigate the health problems of patients with inherited bleeding disorders during the coronavirus disease 2019 pandemic. Material and Methods: Children and adult patients with inherited bleeding disorders who had a coronavirus disease 2019 infection between March 2020 and November 2021 were retrospectively evaluated. Results: Seven hundred seventy-two patients were reviewed, and 65 patients who had a coronavirus disease 2019 infection (Male/Female: 58/7, mean age 28.2 ±14.1 years) were analyzed. Sixty patients (92%) had hemophilia A or B or von Willebrand's disease and 5 (8%) had rare bleeding disorders. Sixteen (24.6%) patients had a comorbid disease and 6 (9.2%) needed hospitalization due to severe coronavirus disease 2019 infection. Seven patients (10.7%) experienced a bleeding episode and were treated with factor concentrates. Totally, 64 (98.46%) patients recovered from the coronavirus disease 2019 infection and 1 died. Conclusion: Patients with inherited bleeding disorders and coronavirus disease 2019 infection mostly had a mild/moderate course of the disease. [ABSTRACT FROM AUTHOR]

Objective: Factor VIII (FVIII) prophylaxis is now considered a gold standard for the treatment of hemophilia A patients to prevent bleeding and maintain musculoskeletal function. FVIII prophylaxis, tailored with pharmacokinetic data, is the most successful approach in preventing bleeding and reducing the cost of treatment. Material and Methods: In this study, 17 patients with severe hemophilia A (median age 11.5 years, median factor 25 U/kg) whose FVIII pharmacokinetic data were obtained through the myPKFiT web access program, were presented. Results: The mean FVIII half-life of the patients was 9.1 hours (min-max: 6.8-11.4); the mean FVIII clearance was 4.7 ml/hour/kg (min-max: 2.6-6.8); and the mean duration of FVIII levels under 1% was 47 hours (min-max: 33-61). The half-life of FVIII was increased with the age of the patient. When a weekly dose schedule simulated with the target FVIII trough level above 1%, it was observed that the dose of FVIII was sufficient in three, it was high in five, and it was insufficient in nine patients. Conclusion: In our study, prophylaxis doses and frequencies were adjusted according to the FVIII pharmacokinetic data. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the causes of thrombocytosis, which was defined as a platelet count greater than 500 x 109/L, and to compare the groups with mild and severe thrombocytosis. A total of 484 patients were evaluated for the etiology of thrombocytosis. Patients with a platelet count between 500-800 x 109/L were considered to have mild thrombocytosis, while those with a count of ≥800 x 109/L were considered as having severe thrombocytosis. Of 484 patients included, 63% had thrombocytosis due to an infectious disease, 11.4% had a chronic inflammatory condition, 8.5% had anemia, and 5.2% had tissue injury. The frequency of chronic inflammation was higher in the severe thrombocytosis group compared to the mild thrombocytosis group (p=0.006). In conclusion, severe infections and chronic inflammatory conditions should be considered in the differential diagnosis of a patient with severe thrombocytosis. [ABSTRACT FROM AUTHOR]

Use of activated prothrombin complex concentrate (aPCC) and recombinant factor VIIa (rFVIIa) is indicated for the treatment and prophylaxis of hemophiliac patients who has developed inhibitors. rFVIIa is an off labeled drug for prophylaxis in Turkey and not covered by health insurance. In this manuscript, the efficacy and dosage for aPCC prophylaxis were evaluated. During the aPCC prophylaxis, bleeding did not occur in any of the three cases. However, two patients developed recurrent bleeding attacks after the prophylaxis ceased. Therefore, we suggest that prophylaxis by aPCC should be prolonged to prevent recurrent bleeding attacks in hemophiliacs who developed inhibitors. [ABSTRACT FROM AUTHOR]

Copyright of Turkish Journal of Hematology is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Turkish Journal of Hematology is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background and Objectives: Acute promyelocytic leukemia (APL), is a distinct subtype of acute myeloid leukemia (AML) characterized by a tendency to hemorrhage and excellent response to all-trans retinoic acid (ATRA). In this retrospective study, we aimed to determine the incidence, clinical symptoms, toxicities, and outcome of children with APL in our center., Methods: We retrospectively reviewed the medical records of children (age < 18 years) diagnosed with APL in our pediatric hematology department between January 2006-December 2016., Results: Pediatric APL represents 20.5% of AML cases in this cohort. Most of the cases presented as classical M3, albeit hypogranular variant was described in 12% of the cohort. Patients with hypogranular variant APL were differed from classical APL by co-expression of CD2 and CD34. About ¾ of APL patients had hemorrhagic findings at admission or the induction treatment. Severe bleeding manifested as intracranial hemorrhage was present in three patients and intracranial arterial thrombosis was present in one. Six patients showed side effects of ATRA such as pseudotumor cerebri, differentiation syndrome resulting in dilated cardiomyopathy, and pulmonary infiltrates. Five-year overall survival (OS) and early death rate were found to be 82.5% and 12% respectively., Conclusions: A high frequency (20.5%) of APL was noted among children with AML in this single-center study. The overall mortality rate was 17.5%. Since the induction death rate was 12% and life-threatening bleeding was the primary problem, awareness and urgent treatment are critical factors to reduce early losses., Competing Interests: Competing interests: The authors have declared that no competing interests exist.

This study evaluated the diagnostic superiority of serum transferrin receptor (sTfR) measurement to other laboratory tests performed for the determination of iron deficiency caused by chronic disease (CD). Study group consisted of 114 anemic patients allocated into 3 groups at the pediatrics clinic of Ankara University, Faculty of Medicine and 32 healthy pediatric subjects as the control group. sTfR value ranged between 11.00 nmol/L and 26.20 nmol/L for the control group. However, it was 61.29 ± 39.33 nmol/L in iron deficiency anemia (IDEA) group, consisting of 51 patients. While there was a significant positive correlation between sTfR and Hb, MCV, serum iron, ferritin levels, there was a significantly inverse correlation between sTfR with RDW in the IDEA group. Mean sTfR value was found 47.05 ± 28.07 nmol/L in the acute infection (AI) group consisting of 22 patients, 39.31 ± 26.16 nmol/L in the CD group with 41 patients. Statistically significant difference was found between sTfR of IDEA group and sTfR of the AI group, though accompanied by iron deficiency. These findings showed that sTfR levels were suppressed. That all hematologic parameters including ferritin and sTfR of all patients in the CD group were heterogenous and revealed both normal and abnormal values was noteworthy. To determine the existence of accompanying iron deficiency, sTfR values above 28.10 nmol/L were chosen. It was concluded that the diagnosis of these patients was not possible without sTfR measurement. TfR-F index (sTfR/log ferritin) was found 97.72 ± 108.81 in the IDEA group, 52.16 ± 66.25 in the AI group, and 24.36 ± 43.00 in the CD group (p< 0.001). TfR-F index values of the chronic disease anemia group (ACD) and the iron deficiency anemia group accompanied by chronic disease (COMBI) were compared. The difference between these two groups was statistically significant (p< 0.001). Thus, TfR-F index proved useful in evaluating the changes of iron metabolism and reducing iron necessities in patients.