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  • Küçük Resim
    Öğe
    Clinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: a multicenter experience
    (Springer, 2021) Şahin, Deniz Gören; Akay, Olga Meltem; Keklik, Muzaffer; Okan, Vahap; Karakuş, Abdullah; Demir, Cengiz; Erkurt, Mehmet Ali; İlkkılıç, Kadir; Yıldırım, Rahşan; Çağlıyan, Gülsüm Akgün; Aksu, Salih; Doğu, Mehmet Hilmi; Dal, Mehmet Sinan; Karakuş, Volkan; Gemici, Ali İhsan; Terzi, Hatice; Kelkitli, Engin; Şıvgn, Serdar; Ünal, Ali; Yılmaz, Mehmet; Ayyıldız, Orhan; Korkmaz, Serdal; Eser, Bülent; Altuntaş, Fevzi
    The aim of this study is to collect paroxysmal nocturnal hemoglobinuria (PNH) patient data from hematology centers all over Turkey in order to identify clinical features and management of PNH patients. Patients with PNH were evaluated by a retrospective review of medical records from 19 different institutions around Turkey. Patient demographics, medical history, laboratory findings, and PNH-specific information, including symptoms at the diagnosis, complications, erythrocyte, and granulocyte clone size, treatment, and causes of death were recorded. Sixty patients (28 males, 32 females) were identified. The median age was 33 (range; 17-77) years. Forty-six patients were diagnosed as classic PNH and 14 as secondary PNH. Fatigue and abdominal pain were the most frequent presenting symptoms. After eculizumab became available in Turkey, most of the patients (n = 31/46, 67.4%) were switched to eculizumab. Three patients with classic PNH underwent stem cell transplantation. The median survival time was 42 (range; 7-183 months) months. This study is the first and most comprehensive review of PNH cases in Turkey. It provided us useful information to find out the differences between our patients and literature, which may help us understand the disease.
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    Öğe
    Idelalisib at the Crossroads of B-Cell Lymphoproliferative Disorders
    (Akad Doktorlar Yayinevi, 2016) Aksu, Salih; Ayyildiz, Orhan; Etgul, Sezgin; Goker, Hakan; Gunes, Gursel; Haznedaroglu, Ibrahim C.; Ilhan, Osman
    Phosphatidylinositol 3-kinases (PI3Ks) are considered as lipid kinases that are very active in the pathobiology of lymphoproliferative disorders (LPDs). Idelalisib, a selective inhibitor of the delta isoform of PI3K, provides significant clinical efficacy and has an acceptable side-effect profile in the treatment of B-LPDs. The aim of this review is to outline the pharmacobiological basis of idelalisib that is located at the crossroads of B-LPDs. The PI3K signaling pathway with downstream targets including Akt is involved in hematologic malignancies and lymphomas. Idelalisib has been most widely studied in chronic lymphoid leukemia (CLL) and B-lymphoma. The activity of idelalisib in high-risk FL with early relapse following front line immunochemotherapy was recently shown. The unique immunological toxicity pattern of idelalisib was also decribed in this review. Further clinical investigations will help for the better selection of the subsets of the patients with B-LPD that would be best candidates for the clinical utilization of idelalisib. Other indications such as marginal zone lymphoma, mantle cell lymphoma, Waldenstrom's Macroglobulinemia and other B-cell disorders could likely to be expanded. Future clinical and experimental data combined with the next-generation genomics strategies and personalized medicine for the treatment of malignant disorders will enlightened us for better placement of idelalisib in the treatment algorithm of the patients.