Browsing by Author "Patiroglu, Turkan"

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The Association Between Hla Class I and Ii Alleles and The Occurrence of Inhibitors in Turkish Patients With Hemophilia A: a Pilot Study
(Wiley, 2019) Patiroglu, Turkan; Cansever, Murat; Akbayram, Sinan; Gulen, Huseyin; Oncel, Kahraman; Borst, Ozcan; Kavakli, Kaan
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)
Clinical and Molecular Aspects of Turkish Familial Hemophagocytic Lymphohistiocytosis Patients With Perforin Mutations
(Pergamon-elsevier Science Ltd, 2008) Okur, Hamza; Balta, Gunay; Akarsu, Nurten; Oner, Ahmet; Patiroglu, Turkan; Bay, Ali; Gurgey, Aytemiz
The aim of this study was to elucidate the pathologic sequence changes and associated clinical phenotypes in 9 new patients showing homozygosity for perforin gene among a total of 37 (24%) Turkish FHL families studied by linkage analysis. These 9 unrelated patients (5M/4F) were coming from consanguineous families and their presentation ages of systemic symptoms were ranged from birth to 15 years. Direct sequencing of coding exons of the perforin gene led to the identification of five different homozygous alterations. The nonsense W374X mutation was identified in three patients while four different missense mutations namely G149S, V50M, A91V and novel A523D were detected in the rest six patients. (c) 2007 Elsevier Ltd. All rights reserved.
Familial Hemophagocytic Lymphohistiocytosis Type Iv: Possible Founder Effect in Syntaxin 11 Gene Common Mutation Endemic To Turkish Population
(Amer Soc Hematology, 2008) Caliskan, Umran; Guler, Elif; Patiroglu, Turkan; Oner, Ahmet F.; Gurgey, Aytemiz