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    Eltrombopag for the Treatment of Immune Thrombocytopenia: The Aegean Region of Turkey Experience
    (Galenos Yayincilik, 2015) Ozdemirkiran, Fusun; Payzin, Bahriye; Kiper, H. Demet; Kabukcu, Sibel; Cagliyan, Gulsum Akgun; Kahraman, Selda; Sevindik, Omur Gokmen; Ceylan, Cengiz; Kadikoylu, Gurhan; Sahin, Fahri; Keskin, Ali; Arslan, Oyku; Ozcan, Mehmet Ali; Gorgun, Gulnur; Bolaman, Zahit; Buyukkececi, Filiz; Bilgir, Oktay; Alacactoglu, Inci; Vura, Filiz; Tombuloglu, Murat; Gokgoz, Zafer; Saydam, Güray
    Objective: Immune thrombocytopenia (ITP) is an immune-mediated disease characterized by transient or persistent decrease of the platelet count to less than 100x109/L. Although it is included in a benign disease group, bleeding complications may be mortal. With a better understanding of the pathophysiology of the disease, thrombopoietin receptor agonists, which came into use in recent years, seem to be an effective option in the treatment of resistant cases. This study aimed to retrospectively assess the efficacy, long-term safety, and tolerability of eltrombopag in Turkish patients with chronic ITP in the Aegean region of Turkey. Materials and Methods: Retrospective data of 40 patients with refractory ITP who were treated with eltrombopag in the Aegean region were examined and evaluated. Results: The total rate of response was 87%, and the median duration of response defined as the number of the platelets being over 50x10(9)/L was 19.5 (interquartile range: 5-60) days. In one patient, venous sinus thrombosis was observed with no other additional risk factors due to or related to thrombosis. Another patient with complete response and irregular follow-up for 12 months was lost due to sudden death as the result of probable acute myocardial infarction. Conclusion: Although the responses to eltrombopag were satisfactory, patients need to be monitored closely for overshooting platelet counts as well as thromboembolic events.
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    Ischemia-Modified Albumin Levels in Essential Thrombocytosis
    (2018) Ersoy, Ercan; Soyaltın, Utku Erdem; Peker, Ahmet; Çolak, Ayfer; Ceylan, Cengiz; Akar, Harun
    Objective: the aim of this study was to investigate the levels of ischemia-modified albumin (IMA) in people with essential thrombocytosis (ET). Methods: A total of 30 patients with ET patient group and 30 volunteers with no known disease control group (C group) were included in this study after the approval of ethics committee and written informed consent was obtained. Patients with a history of major thrombosis were excluded. IMA levels and independent variables were investigated and effects on thrombosis susceptibility were also studied. in addition to that; comorbid disease state, drug use and used drugs group were questioned in patient group and effects of them on IMA levels were studied. Results: in our study, when ET patient group and C group were compared, the mean serum IMA levels in ET patient group and C group was detected as 0,6726 (0,527- 0,776) Absorbans Unite (ABSU) and 0,4342 (0,346-0,612) ABSU respectively and in ET patient group was significantly higher than C group (p<0,001). the glucose, total cholesterol and triglyceride values were significantly higher in ET patient group (p= 0,026, p= 0,058, p= 0,004, respectively). There is a correlation between IMA concentration and age (p= 0,042). Conclusions: in our study IMA levels were found significantly high in ET patient group, this supports increased risk of thrombosis in ET. the difference of metabolic parameters between the ET patient group and C group can be explained by insulin resistance and atherosclerosis background caused by chronic inflammation.
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    Ischemia-Modified Albumin Levels in Essential Thrombocytosis
    (Duzce Univ, 2018) Ersoy, Ercan; Soyaltin, Utku Erdem; Peker, Ahmet; Colak, Ayfer; Ceylan, Cengiz; Akar, Harun
    Objective: The aim of this study was to investigate the levels of ischemia-modified albumin (IMA) in people with essential thrombocytosis (ET). Methods: A total of 30 patients with ET patient group and 30 volunteers with no known disease control group (C group) were included in this study after the approval of ethics committee and written informed consent was obtained. Patients with a history of major thrombosis were excluded. IMA levels and independent variables were investigated and effects on thrombosis susceptibility were also studied. In addition to that; comorbid disease state, drug use and used drugs group were questioned in patient group and effects of them on IMA levels were studied. Results: In our study, when ET patient group and C group were compared, the mean serum IMA levels in ET patient group and C group was detected as 0,6726 (0,527-0,776) Absorbans Unite (ABSU) and 0,4342 (0,346-0,612) ABSU respectively and in ET patient group was significantly higher than C group (p<0,001). The glucose, total cholesterol and triglyceride values were significantly higher in ET patient group (p= 0,026, p= 0,058, p= 0,004, respectively). There is a correlation between IMA concentration and age (p= 0,042). Conclusions: In our study IMA levels were found significantly high in ET patient group, this supports increased risk of thrombosis in ET. The difference of metabolic parameters between the ET patient group and C group can be explained by insulin resistance and atherosclerosis background caused by chronic inflammation.
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    Multidisciplinary clinical management of paroxysmal nocturnal hemoglobinuria
    (E-Century Publishing Corp, 2015) Sahin, Fahri; Ozkan, Melda Comert; Mete, Nihal Gokmen; Yilmaz, Mumtaz; Oruc, Nevin; Gurgun, Alev; Kayikcioglu, Meral; Guler, Ayse; Gokcay, Figen; Bilgir, Ferda; Ceylan, Cengiz; Bilgir, Oktay; Sari, Ismail Hakan; Saydam, Güray
    Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease caused by clonal expansion of one or more hematopoietic stem cell (HSC) lines due to a somatic mutation of the phosphatidylinositol glycan anchor (PIG-A) gene located on Xp22.1. PNH incidence is 1.5-2 cases per million of the population per year. PNH can affect multiple systems in the body and requires multidisciplinary clinical management. Patients can manifest with severe pancytopenia, life-threatening thrombosis affecting the hepatic, abdominal, cerebral, and subdermal veins, and high requirements for blood transfusion due to haemolytic anemia. PNH can also be associated with bone marrow failure. Advances in diagnostic techniques and a targeted therapeutic approach for PNH have emerged in the last two decades. Eculizumab, a promising humanized monoclonal antibody against C5, is the first approved therapy for PNH.
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    Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria
    (Hindawi Ltd, 2015) Patir, Pusem; Isik, Yakup; Turk, Yigit; Ugur, Mehmet Can; Ceylan, Cengiz; Gorgun, Gulnur; Gokmen, Nihal Mete; Saydam, Güray; Sahin, Fahri
    Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of infections due to complement-associated defects subsequent to CD59 deficiency. Here, we report a rare case of a 65-year-old male patient with necrotic ulcers on both legs, where the recognition of pancytopenia and microthrombi led to the diagnosis of PNH based on FLAER (FLuorescent AERolysin) flow cytometric analysis. He was subsequently started on eculizumab therapy, with starting and maintenance doses set as per drug labelling. Progression of the patient's leg ulcers during follow-up, with fulminant tissue destruction, purulent discharge, and necrotic patches, led to a later diagnosis of necrotizing fasciitis due to Pseudomonas aeruginosa and Klebsiella pneumonia infection. Courses of broad-spectrum antibiotics, surgical debridement, and superficial skin grafting were applied with successful effect during ongoing eculizumab therapy. This case highlights the point that it is important to maintain treatment of underlying disorders such as PNH in the presence of life-threatening infections like NF.
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    Prognostic impact of next-generation sequencing on myelodysplastic syndrome: A single-center experience
    (Lippincott Williams & Wilkins, 2024) Bulbul, Hale; Kaya, Ozge Ozer; Karadag, Fatma Keklik; Olgun, Aybuke; Demirci, Zuhal; Ceylan, Cengiz
    Myelodysplastic syndromes (MDS) are clinically heterogeneous disorders characterized by peripheral blood cytopenias, poor differentiation, clonal hematopoiesis, and increased risk of developing acute myeloid leukemia (AML). While somatic mutations do not currently feature in prognostic scoring systems, they may impact the clinical phenotype. In recent years, next-generation sequencing (NGS) has enabled the opportunity to identify an increasing number of genetic abnormalities, including recurrent modifications in the TP53, DNMT3A, NRAS, NPM1, RUNX1, and FLT3 genes. Bone marrow aspirate samples of 56 patients with MDS were investigated for mutations using NGS. We compared the relationship between gene mutation status and laboratory characteristics, such as certain cytopenias, the revised international prognostic scoring system, MDS subtypes, karyotypes, AML development, and overall survival. Twenty-one genes were found to have gene mutations, including ASXL1, TET2, SRSF2, EZH2, CSF3R, NRAS, ETV6, SETBP1, RUNX1, DDX41, U2AF1, JAK2, FLT3ITD, SF3B1, DNAMT3A, PHF6, TP53, CEBPA, CBL, IDH2, and GATA2. At least one point mutation occurred in 64.2% of all patients, including 58.3% of those with normal cytogenetics. Thrombocytopenia (P = .016), anemia (P = .018), decreased overall survival (P = .017), and increased AML transformation (P = .023) have been revealed to be linked to non-SF3B1 mutations. MDS are frequently associated with somatic point mutations. According to early findings, NGS panels are extremely effective instruments that provide an entirely new viewpoint on the disease for particular individuals. Future prognostications will depend more on NGS because those who exhibit normal cytogenetics may additionally have gene mutations.