Myelodisplastik Sendrom Tanılı Olguların Sitogenetik / Fish ve Demografik Verilerinin İncelenmesi

Amaç: Myelodisplastik sendrom (MDS) kemik iliğinde anormal selüler proliferasyon, çevre kanında, bir veya daha fazla hücre dizisinde sitopeni, kemik iliğinde displazi ve akut myeloid lösemi (AML) gelişme riski ile karakterize heterojen bir hastalık grubudur. Bu çalışmanın amacı MDS hastalarında demografik veriler, konvansiyonel sitogenetik, Florasan in situ hibridizasyon (FİSH) ve prognostik özelliklerini değerlendirmektir.Yöntemler: Bu çalışmaya Ocak 2010 - Ocak 2020 tarihleri arasında DEÜTF Hematoloji Bölümü’nde takip edilen 18 yaş ve üzerindeki MDS tanılı hastalar dahil edildi. Hastaların verileri geriye dönük arandı. Herhangi bir zamanda tanı alan hastaların hemogram, biyokimya, sitogenetik ve FİSH sonuçları, sağ kalımları, almış oldukları tedaviler, sitopeni dereceleri, kemik iliği blast yüzdeleri, kemik iliği biyopsi sonuçları incelendi.Bulgular: Çalışmaya Ocak 2010-Ocak 2020 tarihleri arasında MDS tanısı almış ve izlemde olan 18 yaş ve üzerindeki 205 hasta alındı. Kadın/Erkek oranı 0,8/1 olarak bulundu. Ortanca yaş 70,7 (27-92) idi. Ortanca hemoglobin değeri 9,4 (4.8-14.5) idi. MDS olguları, 2016 Revize-WHO Sınıflaması’na göre sınıflandırıldıklarında; en fazla 78 hasta (%38) ile MDS-SLD grubunda saptandı. Konvansiyonel sitogenetik analiz yapılabilen 141 hastanın 112’sinde (%79,4) normal karyotip saptandı ve bu yöntem ile en fazla saptanan sitogenetik anomali komplex karyotipti (n=9, %6,3). FİSH paneli ile bakılan 135 hastanın genetik anomalilerine göre medyan sağkalımlarına bakıldı. FİSH sonucuna göre 7q delesyonu ve P53 mutasyonu olan hasta popülasyonlarında, mutasyon olmayan gruba göre azalmış ortanca sağkalım süreleri istatistiksel olarak anlamlı saptanmıştır (p-değeri &lt;0,05). Kemik iliğinde CD34 yüzdesi artıkça sağkalımın anlamlı şekilde azaldığı saptandı. Hastaların ortanca sağ kalımları İPSS evresine göre değerlendirildiğinde, ileri evrede bulunan hastaların medyan sağkalımları istatistiksel olarak anlamlı derecede daha düşüktü.Sonuç: MDS’in genetik temeline ilişkin yapılan çalışmalar hastalığın tanı, evreleme, prognoz ve tedavi yöntemlerini şekillenmesinde önemli rol oynamaktadır.Objective: A myelodysplastic syndrome (MDS) is a heterogeneous group of diseases characterized by abnormal cellular proliferation in the bone marrow, cytopenia in one or more cell lines in the peripheral blood, dysplasia in the bone marrow, and risk of developing acute myeloid leukemia (AML).This study aims to evaluate demographic data, conventional cytogenetics, Fluorescent in situ hybridization (FISH), and prognostic features in MDS patients.Methods: Patients aged 18 years and over 18 years of age with MDS who were followed up in the DEUTF Hematology Department between January 2010 and January 2020 were included in this study. The data of the patients were searched retrospectively. Hemogram, biochemistry, cytogenetic and FISH results, survival, treatments, cytopenia grades, bone marrow blast percentages, bone marrow biopsy results of patients diagnosed at any time were examined.Results: A total of 205 patients over the age of 18 diagnosed with MDS and followed up between January 2010 and January 2020 were included in the study. The female/male ratio was found to be 0.8/1. The median was 70.7 (27-92). The median hemoglobin value was 9.4 g/dl (4.8-14.5). MDS cases are classified according to the 2016 Revised-WHO Classification; it was detected in the MDS-SLD group with the highest 78 patients (38%). A normal karyotype was found in 112 (79.4%) of 141 patients for whom conventional cytogenetic analysis could be performed, and the most common cytogenetic anomaly detected with this method was complex karyotype (n=9, 6.3%). The median survival of 135 patients with the FISH panel was evaluated according to their genetic anomalies. According to the FISH result, in the patient populations with 7q deletion and P53 mutation, decreased median survival times were statistically significant compared to the group without mutation (p-value &lt;0.05). It was determined that survival decreased significantly as the percentage of CD34 increased in the bone marrow. When the median survival of the patients was evaluated according to the IPSS stage, the median survival of the patients in the advanced stage was statistically significantly lower.Conclusion: Studies on the genetic basis of myelodysplastic syndrome play an essential role in shaping the diagnosis, staging, prognosis, and treatment methods of the disease.</p

Berlin-Frankfurt-Munster (BFM) versus Hyperfractionated Cyclophosphamide, Vincristine, Doxorubicin, Dexamethasone (Hyper-CVAD) chemotherapy regimens in Adult T-Cell ALL and Adolescent and Young Adult (AYA) Patients: A Retrospective , Comperative Evaluation and Review

Objectives: Recently, in adult ALL patients, to improve prognosis, pediatric ALL protocols have been used in adolescent and young adults and positive results have been reported.Methods: In this study, we aimed to evaluate retrospectively the demographic features, treatment responses, cytogenetic data and survival analyses of adult T-All cases followed up by the Department of Adult Hematology at Dokuz EylulUniversity Hospital.Results: The mean survival time for patients receiving H-CVAD was 46.9 months (±12.9), and median survival time ofpatients receiving BFM treatment was 71.5 months (±11.4).The median OS of patients receiving BFM was statisticallysignificantly longer than that of Hyper-CVAD (p=0.044).Conclusion: The use of pediatric intensive chemotherapy regimens in adolescent and young adult (AYA) patients withlow incidence and poor prognosis of ALL-type T-ALL, the inclusion of nelarabine in the first line treatment and the selection of new targeted therapies based on new genomic discoveries can increase effectiveness. The use of pediatricintensive chemotherapy regimens is promising for increased survival in adults</p