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    The Association Between JAK2V617F Mutation and Bone Marrow Fibrosis at Diagnosis in Patients with Philadelphia-Negative Chronic Myeloproliferative Neoplasms
    (Galenos Yayincilik, 2012) Ar, M. Cem; Buyuktas, Deram; Eskazan, A. Emre; Aydin, Seniz Ongoren; Tanrikulu, Eda; Baslar, Zafer; Buyru, A. Nur
    Objective: Bone marrow fibrosis is the second most common complication that causes morbidity and mortality in patients with Philadelphia-negative myeloproliferative neoplasms (MPNs). The aim of this study was to investigate the association between JAK2V617F mutation and bone marrow fibrosis at diagnosis in patients with MPNs. Material and Methods: In total, 149 patients with MPNs were retrospectively evaluated to determine if there was an association between the histological grade of bone marrow fibrosis and JAK2V617F mutation. Results: In all, 67.7% of the patients carried the mutated JAK2 gene. The presence of JAK2V617F mutation was not associated with the occurrence of bone marrow fibrosis (P = 0.55) or its grade at diagnosis (P = 0.65). Conclusion: Molecular mechanisms or genetic defects other than JAK2V617F may underlie the occurrence of bone marrow fibrosis in patients with MPNs.
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    Intracranial extramedullary hematopoiesis in patients with thalassemia: a case report and review of the literature
    (Wiley, 2012) Eskazan, Ahmet Emre; Ar, Muhlis Cem; Baslar, Zafer
    BACKGROUND: Extramedullary hematopoiesis (EH) is a compensatory phenomenon that results in the production of blood cell precursors outside the marrow in patients with chronic hemolytic anemia and ineffective erythropoiesis. EH usually involves the liver, spleen, and lymph nodes. It can also be found at paravertebral, intrathoracic, or pelvic locations. Intracranial EH is a rare entity and often asymptomatic but can sometimes lead to symptomatic tumor-like masses. Treatment options are controversial and include hypertransfusion, surgical excision, radiotherapy, and hydroxyurea (HU). STUDY DESIGN AND METHODS: Successful treatment of an intracranial EH mass with HU and blood transfusions in a beta-thalassemia major patient was discussed along with a review of the published literature on intracranial EH in thalassemia. RESULTS: In our patient, the extramedullary hematopoietic mass in the interhemispheric fissure showed a marked improvement after 6 months of HU and hypertransfusion therapy. In the English literature, there are a few cases with intracranial EH and thalassemia, which were treated with different treatment modalities, with different outcomes. CONCLUSION: There is no standard treatment approach in patients with symptomatic EH. HU with hypertransfusion regimen is a reasonable first-choice modality in treating intracranial EH masses.