Browsing by Author "Celkan, Tiraje"

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Clinical and Laboratory Data of Primary Hemophagocytic Lymphohistiocytosis: a Retrospective Review of the Turkish Histiocyte Study Group
(Galenos Yayincilik, 2010) Fisgin, Tunc; Patiroglu, Turkan; Ozdemir, Akif; Celkan, Tiraje; Caliskan, Umran; Ertem, Mehmet; Karapinar, Deniz Yilmaz
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. (Turk J Hematol 2010; 27: 157-62)
Use of a High-Purity Factor X Concentrate in Turkish Subjects With Hereditary Factor X Deficiency: Post Hoc Cohort Subanalysis of a Phase 3 Study
(Galenos Yayincilik, 2018) Oner, Ahmet F.; Celkan, Tiraje; Timur, Cetin; Norton, Miranda; Kavakli, Kaan
Hereditary factor X (FX) deficiency is a rare bleeding disorder more prevalent in countries with high rates of consanguineous marriage. In a prospective, open-label, multicenter phase 3 study, 25 IU/kg plasma-derived factor X (pdFX) was administered as on-demand treatment or short-term prophylaxis for 6 months to 2 years. In Turkish subjects (n=6), 60.7% of bleeds were minor. A mean of 1.03 infusions were used to treat each bleed, and mean total dose per bleed was 25.38 IU/kg. Turkish subjects rated pdFX efficacy as excellent or good for all 84 assessable bleeds; investigators judged overall pdFX efficacy to be excellent or good for all subjects. Turkish subjects had 51 adverse events; 96% with known severity were mild/moderate, and 1 (infusion-site pain) was possibly pdFX-related. These results demonstrate that 25 IU/kg pdFX is safe and effective in this Turkish cohort