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Objectives To evaluate the long-term efficacy and safety of deferasirox therapy in a large observational cohort of children with transfusion-dependent thalassemia (TDT) and sickle cell anemia (SCA) in Turkey. Methods This was a multicenter, prospective cohort study including TDT and SCA patients aged 2-18 years with iron overload (>= 100 mL/kg of pRBC or a serum ferritin [SF] level >1000 mu g/L) receiving deferasirox. Patients were followed for up to 3 years according to standard practice. Results A total of 439 patients were evaluated (415 [94.5%] TDT, 143 [32.6%] between 2 and 6 years). Serum ferritin levels consistently and significantly decreased across 3 years of deferasirox therapy from a median of 1775.5 to 1250.5 mu g/L (P < 0.001). Serum ferritin decreases were noted in TDT (1804.9 to 1241 mu g/L), SCA (1655.5 to 1260 mu g/L), and across age groups of 2-6 years (1971.5 to 1499 mu g/L), 7-12 years (1688.5 to 1159.8 mu g/L), and 13-18 years (1496.5 to 1107 mu g/L). Serum ferritin decreases were also noted for all deferasirox dose groups but only significant in patients with doses >= 30 mg/kg/d (n = 120, -579.6 median reduction, P < 0.001). Only 9 (2%) patients had adverse events suspected to be related to deferasirox. Serum creatinine slightly increased but remained within the normal range. Conclusions Deferasirox has long-term efficacy and safety in children with TDT and SCA, although higher doses (>= 30 mg/kg/d) may be required to achieve iron balance. C1 [Antmen, Bulent] Acibadem Hosp, Adana, Turkey. [Karakas, Zeynep] Istanbul Univ, Med Fac, Istanbul, Turkey. [Yesilipek, Mehmet Akif; Kupesiz, Osman Alphan] Akdeniz Univ, Med Fac, Antalya, Turkey. [Sasmaz, Ilgen] Cukurova Univ, Med Fac, Adana, Turkey. [Uygun, Vedat; Kurtoglu, Erdal] Antalya Training & Res Hosp, Antalya, Turkey. [Oktay, Gonul] Antakya State Hosp, Antakya, Turkey. [Aydogan, Gonul; Salcioglu, Zafer] Kanuni Sultan Suleyman Training & Res Hosp, Istanbul, Turkey. [Akin, Mehmet] Denizli State Hosp, Denizli, Turkey. [Vergin, Canan] Dr Behcet Uz Child Dis Surg Training & Res Hosp, Izmir, Turkey. [Kazanci, Elif Guler] Dortcelik Child Dis Hosp, Bursa, Turkey. [Unal, Selma] Mersin Univ, Med Fac, Mersin, Turkey. [Caliskan, Umran] Necmettin Erbakan Univ, Meram Med Fac, Konya, Turkey. [Aral, Yusuf Ziya] Adnan Menderes Univ, Med Fac, Aydin, Turkey. [Turkkan, Emine] Okmeydani Training & Res Hosp, Istanbul, Turkey. [Gunes, Adalet Meral] Uludag Univ, Med Fac, Bursa, Turkey. [Tunc, Bahattin] Hematol Oncol Training & Res Hosp, Ankara Child Hlth & Dis, Ankara, Turkey. [Gumruk, Fatma] Hacettepe Univ, Med Fac, Ankara, Turkey. [Ayhan, Aylin Canbolat; Timur, Cetin] Goztepe Training & Res Hosp, Istanbul, Turkey. [Soker, Murat] Dicle Univ, Med Fac, Diyarbakir, Turkey. [Koc, Ahmet; Oymak, Yesim] Harran Univ, Med Fac, Sanliurfa, Turkey. [Ertem, Mehmet] Ankara Univ, Fac Med, Ankara, Turkey. [Yildirmak, Yildiz] Sisli Etfal Training & Res Hosp, Istanbul, Turkey. [Irken, Gulersu] Dokuz Eylul Univ, Med Fac, Izmir, Turkey. [Apak, Hilmi] Istanbul Univ, Cerrahpasa Med Fac, Istanbul, Turkey. [Biner, Betul; Eren, Tugba Gurleyen] Trakya Univ, Med Fac, Edirne, Turkey. [Balci, Yasemin Isik] Pamukkale Univ, Med Fac, Denizli, Turkey. [Kocak, Ulker] Gazi Univ, Med Fac, Ankara, Turkey. [Karasu, Gulsun] Istanbul Zeynep Kamil Women & Childrens Dis Raini, Istanbul, Turkey. [Akkaynak, Diyar] Novartis Saglik Gida & Tarim Urunleri San & Tic A, Istanbul, Turkey. [Patiroglu, Turkan] Erciyes Univ, Med Fac, Kayseri, Turkey.

22nd Congress of the European-Hematology-Association -- JUN 22-25, 2017 -- Madrid, SPAIN, WOS: 000404127006027, European Hematol Assoc

European Society for Blood and Marrow Transplantation

European Society Blood and Marrow Transplantation

PubMedID: 22931438 From January 1991 to June 2009, 245 children with beta thalassemia major who underwent their first allogeneic HSCT in Turkey and who were followed for a minimum of one yr post-transplantation were enrolled this study. The median age of the patients was 6.6 yr old (range, 1-22 yr). The distribution of Pesaro risk class I, II, and III categories was 41, 130, and 63 children, respectively. The median serum ferritin level was 2203 ng/mL. Eighty-eight patients received bone marrow (BM) stem cells; 137, peripheral blood (PB) stem cells; and 20, cord blood (CB) stem cells. The donors were HLA-matched siblings or parents. Median engraftment times were shorter in PBSCT patients compared with the BMT group (p < 0.001). Grade II-IV acute GvHD was observed in 33 children (13.5%), while cGvHD was observed in 28 patients (12.5%), eight of whom had the extensive form. Thalassemic reconstitution was observed in 43 (17%) of the transplant patients. Post-transplant aplasia occurred in three patients, and the TRM rate was 7.75%. Seventeen patients were lost after 100 days. The thalassemia-free survival and OS rates were 68% (95% CI, 61.8-74.2) and 85.0% (95% CI, 80.2-89.8), respectively. We believe that this study is important because it is the first multicenter national data for children with beta thalassemia major receiving HSCT. © 2012 John Wiley & Sons A/S.

Objective: This study analyzes the clinical and laboratory findings of children with primary hemophagocytic lymphohistiocytosis (HLH) followed in various referral centers of Turkey. Materials and Methods: A simple three-page questionnaire prepared by the Turkish Histiocyte Study Group was used for documentation of patient data. Results: Age at diagnosis varied from 0.6 to 78 months (median±SD, 16.5±26.1). Sex distribution was almost equal (F/M=10/12). The frequencies of parental consanguinity and sibling death in the family history were 100% and 81.1%, respectively. The most common clinical findings were hepatomegaly (100%) and fever (95%). The most common laboratory findings were anemia (100%), hyperferritinemia (100%) and thrombocytopenia (90.9%). Triglyceride and total bilirubin levels in the deceased versus surviving group appear to be high (triglyceride: 394±183 mg/dl, 289±7 mg/dl; total bilirubin: 2.7±6.9 mg/dl, 0.5±1.2 mg/ dl, respectively). Conclusion: We concluded that fever, hepatosplenomegaly, anemia, thrombocytopenia, and hyperferritinemia are the most common clinical and laboratory findings in primary HLH. Increased triglyceride and total bilirubin level at the time of diagnosis might be an indicator of poor prognosis in HLH.

This paper describes the slagging problems resulted from the coal chemistry in various coal-fired electrical power generation plants in Turkey. The fusion temperature test has been used to evaluate the mAng and slagging behaviour of coal ash for years. However, the coal ash fusion temperatures alone are not sufficient to be able to separate the good versus poor performance of coals. Further investigations about the subject revealed that the iron levels in the coal ash and several indexes such as base to acid ratio, slagging factor etc. are also providing good bases for separating the coals that caused problems. The studies using different approaches showed that the chemical and mineral compositions of coal ash determine its melting characteristics and fusion temperature. The correlative equations based on this assumption are still the most widely used techniques for assessing the deposition characteristics and fusion temperature. It also involves the investigation of chemical and mineralogical composition of coal ash samples from Kemerkoy-Yenikoy-Yatagan-Soma coal-fired electrical power plants. The effects of slag indexes causing slagging were calculated after the determination of the ash fusion temperatures. The calculated values were compared with chemical compositions. The results showed that Soma-Yatagan-Yenikoy coal fly ash holds a tendency of high capacity slagging. Kemerkoy coal ash samples have less slagging tendency compared to the others. This is most probably due to their high acidic alkali content.

Burning of-coal in coal-fired electrical power generation plants produces solid waste in the form of boiler slag and fly ash. A large portion of this production is accounted for the coal fly ash. Coal combustion by-products in Turkey are about 45 million tons per year. This study focuses on the synthesis of zeolites from Soma and Yatagan power plant ashes in laboratory scale and on determining the conditions. which govern the related processes. In an alkaline ambient, fusion and hydrothermal treatment were applied as the test methods. The samples obtained from the experiments were investigated by electron microscope SEM and XRD. The final product was observed and determined to be sodalite, which has very similar crystal structure and properties as zeolite.