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Öğe Allogeneic Hematopoietic Stem Cell Transplantation in Adult Patients with Thalassemia: A Single-Center Experience and Literature Review(2023) Uysal, Ayşe; Erkurt, Mehmet Ali; Sarıcı, Ahmet; Kuku, İrfan; Biçim, Soykan; Hidayet, Emine; Kaya, AhmetObjective: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the only curative treatment modality in thalassemia. Its use has been limited by age, transplant-related mortality (TRM), graft rejection, and graft versus host disease (GvHD), especially in adult patients. We aimed to present our allo-HSCT experience in adult patients with thalassemia major. Material and Method: Patients’ demographic and clinical features, donor types, resource of stem cells, conditioning regimens, GvHD prophylaxis, time to neutrophil and platelet engraftments, acute and chronic GvHD, thalassemia -free survival (TFS) and overall survival were examined. Results: The study included six patients. The median age was 21.5 (20-26) years. The median ferritin levels were 1498.4 (347.4-6992.3) pg/ml. The matched sibling donor (MSD) was used in 4 patients while matched unrelated donor (MUD) was used in 2 patients. The median time to neutrophil and platelet engraftments were 17 (15-35) and 18 (15-40) days, respectively. Acute and chronic GvHD were detected in 2 and 1 patients, respectively. The TRM was detected in 2 patients (33.3%), due to infection and acute GVHD. At a median follow-up of 28 months after transplantation, 4 (66.6%) patients were alive and TFS was achieved in 2 (33.3%) patients. Graft failure was detected in 3 (50%) patients. Conclusion: Graft rejection, TRM and GvHD limited the use of allo-HSCT, especially in adult patients. These complications were reduced by re- duced-intensity conditioning regimens and allo-HSCT should be done primarily in patients under the age of 20 years and without organ damage due to iron overload.Öğe Assessment of left atrial volume and function in patients with Sjogren's syndrome using three-dimensional echocardiography(Wiley, 2020) Hidayet, Siho; Yagmur, Julide; Karaca, Yucel; Bayramoglu, Adil; Yolbas, Servet; Hidayet, Emine; Ulutas, ZeynepObjective We used real time, three-dimensional transthoracic echocardiography (3DTTE) to evaluate left atrial (LA) volume and mechanical function in patients with primary Sjogren's syndrome (SS). Methods We prospectively included 42 consecutive patients with primary SS and 42 controls who were similar in terms of basal characteristics. 3DTTE was used to assess LA function. Results Maximum LA volume, minimum LA volume, pre-atrial contraction LA volume, LA Active Stroke Volume (ASV), LA Total Stroke Volume (TSV), maximal left atrial volume index (LAVImax), Left atrial pre-contraction volume index, and Left atrial minimum volume index, ASV index, and TSV index were significantly higher in the SS group, and the LA Total Emptying Fraction, LA Expansion Index, and LA Passive Emptying Fraction were significantly lower. Although the active emptying fraction was higher in the SS group, the difference was not statistically significant. LAVImax was positive correlated with disease duration (r = .753). Conclusion Left atrial function is impaired in SS patients and serves as an early marker of subclinical cardiac involvement.Öğe Concurrent congenital hemophilia B and acquired hemophilia A: a unique case report(Lippincott Williams & Wilkins, 2024) Cirik, Salih; Erkurt, Mehmet Ali; Kuku, Irfan; Kaya, Emin; Berber, Ilhami; Hidayet, Emine; Bicim, SoykanCongenital hemophilia B is a rare X-linked recessive bleeding disorder caused by factor IX deficiency. Acquired hemophilia A is a rare, acquired bleeding disorder that presents with new-onset bleeding, especially in older adults, due to the development of auto-antibodies against factor VIII (FVIII). This case report presents the medical management of a patient with congenital hemophilia B and acquired hemophilia A. We highlight the limitations of maintaining factor levels with factor replacement therapy alone, particularly in hemophilia patients who have developed factor inhibitors. In addition, we draw attention to the need for dose escalation, the cost, and the need for immune-tolerance induction therapy. This case illustrates that when the current diagnosis does not explain the full clinical picture and laboratory data are inadequate, it is important to continue to seek alternative diagnoses and cost-effective treatment.Öğe Effect of extracorporeal photopheresis on survival in acute graft versus host disease(Wiley, 2023) Kaya, Ahmet; Erkurt, Mehmet Ali; Kuku, Irfan; Kaya, Emin; Berber, Ilhami; Bicim, Soykan; Hidayet, EmineBackground: Extracorporeal photopheresis (ECP) is the main non-pharmacological approach accompanying systemic medical treatments in steroid-resistant acute or chronic graft versus host disease. The study aimed to examine the effect of ECP on survival in acute graft versus host disease (aGVHD).Methods: A total of 35 patients who were followed up in the adult hematology clinic of Inonu University Turgut ozal Medical Center for aGVHD were included in the study. Stem cell transplantation and ECP application parameters that may affect the survival of the patients were examined.Results: In aGVHD using ECP, the degree of involvement affects survival. Involvements with a clinical and laboratory score (Glucksberg system) of 2 and above significantly reduced survival. The duration of ECP use is associated with survival. Especially, 45 days and longer use increases survival (hazard ratio, P-value <.05). The duration of steroid use was found to be effective in survival in aGVHD (P < .001). ECP administration day (P = .003), duration of steroid use (P < .001), duration of ECP use (P = .001), and grade of aGVHD (P < .001) affect survival.Conclusion: ECP use is effective in survival in patients with aGVHD score =2. In patients with aGVHD, especially the use of 45 days and longer has a positive effect on survival. The duration of steroid use is associated with survival in aGVHD.Öğe The effect of G-CSF used after allogeneic hematopoietic stem cell transplantation on engraftment times and platelet suspension replacement numbers(Pergamon-Elsevier Science Ltd, 2022) Sarici, Ahmet; Erkurt, Mehmet Ali; Kuku, Irfan; Kaya, Emin; Berber, Ilhami; Bicim, Soykan; Hidayet, EmineBackground: With the use of granulocyte colony stimulating factor (G-CSF) after allogeneic hematopoietic stem cell transplantation (HSCT), the duration of neutrophil engraftment and hospitalization were shortened. How-ever, there is no consensus on the effect of G-CSF on platelet engraftment time. The primary aim of our study is to determine the effect of G-CSF use on platelet engraftment time after HSCT. Secondary purposes are to determine the number of platelet suspension, number of erythrocyte suspension and incidence of acute graft versus disease after HSCT. Material and methods: Patients who had allogeneic stem cell transplantation at our center between 01.01.2011 and 01.01.2022 were retrospectively analyzed. Patients were divided into 2 groups as those who received and did not receive G-CSF after transplantation. Results: A total of 64 patients were included. While 32 patients were given post-HSCT G-CSF support, the other 32 patients were not given. Neutrophil engraftment time and length of hospital stay were shorter in the group receiving G-CSF (p < 0.05). Platelet engraftment time was shorter in the group that did not receive G-CSF (p < 0.05). The incidence of acute GVHD of the patients in group 1 tended to be higher than the patients in group 2 (40.6 % vs 15.6 %, p = 0.052). Post-HSCT platelet suspension was less in the group that did not receive G-CSF, but this difference was not statistically significant (p = 0.173). Conclusion: While the positive effect of post HSCT G-CSF use on duration of neutrophil engraftment and hospi-talization is evident, its effects on platelet engraftment need to be investigated.Öğe The effect of thrombocytapheresis on hemogram and biochemistry parameters in patients with essential thrombocytemia(Elsevier France-Editions Scientifiques Medicales Elsevier, 2023) Kaya, Ahmet; Kuku, Irfan; Erkurt, Mehmet Ali; Kaya, Emin; Berbera, Ilhami; Bicim, Soykan; Hidayet, EmineBackground: Essential thrombocythemia is one of the chronic myeloproliferative neoplasms characterized by clonal proliferation of myeloid cells with variable morphological maturation and hematopoietic activity. It is characterized by excessive clonal platelet production with a tendency to thrombosis and bleeding. Thrombocytapherests is the removal of platelets by apheresis techniques. Thrombocytapherests is generally recommended in patients with essential thrombocythemia with acute, severe thrombotic or hemorrhagic events.Methods: The study included 39 patients who were diagnosed with essential thrombocythemia, started cytoreductive and aspirin therapy, and underwent thrombocytapherests due to the development of acute severe thrombotic or hemorrhagic events, diagnosed in the adult hematology clinic of I_nonu University Turgut Ozal Medical Center. Hemogram and biochemistry values of the patients were scanned retrospectively.Results: After thrombocytapherests, a statistically significant difference was found between the first and last measurements of hemoglobin, mean platelet volume, White blood cell, neutrophil, platelet, platelet distribution width, creatine, lactate dehydrogenase, fibronogen and calcium levels of the patients. Conclusion: The use of thrombocytapherests in patients with essential thrombocytosis causes a rapid decrease in platelet values as well as an effect on hemogram and biochemistry parameters. Other hemogram and biochemistry parameters such as platelet value should be monitored in patients.(c) 2023 Societe francaise de transfusion sanguine (SFTS). Published by Elsevier Masson SAS. All rights reserved.Öğe Etiological evaluation and the role of plasma exchange treatment in trombotic microangiopathies: A retrospective analysis from Eastern Anatolia(2022) Hidayet, Emine; Erkurt, Mehmet Ali; Sarici, Ahmet; Bicim, Soykan; Berber, Ilhami; Kuku, Irfan; Kaya, Emin; Kaya, Ahmet; Özgül, MustafaThe aim is this study was to make the etiological classification of patients diagnosed with TMA in our region and to statistically evaluate the effect of therapeutic plasma exchange, which we use in treatment, on laboratory recovery and mortality. In our study, between 2009-2017, 85 patients diagnosed with TMA in our center evaluated retrospectively. Thirty- one (36.5%) of our patients were followed up with HELLP, 23 with thrombotic thrombocytopenic purpura (TTP) (27.1%), 20 (23.5%) snake bites, and 11 (12.9%) with atypical hemolytic uremic syndrome (aHUS). TPE treatment was performed in all patients. TPE treatment was found effective in patients with HELLP syndrome, TTP and snake bite and statistically significant improvement was obtained in laboratory parameters (p<0.05). However, TPE treatment was not found to be effective in the treatment of atipical HUS (p>0.05). Mortality rates were found 9.7%, 21.7%, 27.3%, and 0% in patients with HELLP Syndrome, TTP, aHUS, and snake bite, respectively. The primary treatment in HELLP syndrome was termination of pregnancy, it was observed in our study that TPE was effective in TTP, HELLP syndrome and snake bites and treatment should be started without delayÖğe Etiological evaluation and the role of plasma exchange treatment in tromboticmicroangiopathies: A retrospective analysis from Eastern Anatolia(2022) Hidayet, Emine; Erkurt, Mehmet Ali; Sarıcı, Ahmet; Biçim, Soykan; Kuku, İrfan; Berber, İlhami; Kaya, EminThe aim is this study was to make the etiological classification of patients diagnosed with TMA in our region and to statistically evaluate the effect of therapeutic plasma exchange, which we use in treatment, on laboratory recovery and mortality. In our study, between 2009-2017, 85 patients diagnosed with TMA in our center evaluated retrospectively. Thirty- one (36.5%) of our patients were followed up with HELLP, 23 with thrombotic thrombocytopenic purpura (TTP) (27.1%), 20 (23.5%) snake bites, and 11 (12.9%) with atypical hemolytic uremic syndrome (aHUS). TPE treatment was performed in all patients. TPE treatment was found effective in patients with HELLP syndrome, TTP and snake bite and statistically significant improvement was obtained in laboratory parameters (p<0.05). However, TPE treatment was not found to be effective in the treatment of atipical HUS (p>0.05). Mortality rates were found 9.7%, 21.7%, 27.3%, and 0% in patients with HELLP Syndrome, TTP, aHUS, and snake bite, respectively. The primary treatment in HELLP syndrome was termination of pregnancy, it was observed in our study that TPE was effective in TTP, HELLP syndrome and snake bites and treatment should be started without delayÖğe Evaluation of busulfan, cyclophosphamide, and etoposide as a preparation regimen for autologous stem cell transplantation in Hodgkin's lymphoma patients(Wolters Kluwer Medknow Publications, 2024) Bicim, Soykan; Erkurt, Mehmet Ali; Kuku, Irfan; Kaya, Emin; Berber, Ilhami; Kaya, Ahmet; Hidayet, EmineBACKGROUND: Hodgkin's lymphoma (HL) exhibits a cure rate of 90% in patients diagnosed at an early stage and a cure rate ranging from 70% to 90% in patients diagnosed at an advanced stage. In the case of patients with relapsed/refractory HL (r/rHL), it is recommended to provide salvage chemotherapy initially, followed by autologous stem cell transplantation (ASCT). The ideal conditioning regimen for the transplantation process is still being investigated.OBJECTIVES: For individuals with r/rHL, high-dose chemotherapy combined with ASCT (HD-ASCT) is thought to be the most effective method of treatment. The purpose of this research was to evaluate the effectiveness and safety of the busulfan, cyclophosphamide, and etoposide (BuCyE) preparation regimen in r/rHL patients.MATERIALS AND METHODS: Retrospective analysis was conducted on the data of 67 lymphoma patients older than 18 years who had HD-ASCT with the BuCyE conditioning regimen between September 2014 and November 2021 (86 months). The research consisted of 34 r/r HL patients among them. A parenteral regimen of 0.8 mg/kg of busulfan every 6 h from day -7 to day -5, 50 mg/kg of cyclophosphamide on days -3 and -2, and 400 mg/m2 of etoposide on days -5 and -4 comprised the patient preparation regimen before ASCT. All data were collected from inpatient files and the Inonu University Turgut Ozal Medical Center Hospital Information System.RESULTS: The median age of the patients was 43 years, and 67.6% were males. The most common type of HL was nodular sclerosis, which was followed by mixed cellularity. The median time for platelet and neutrophil engraftment was 14 and 11 days, respectively. 5.0 x 106/kg was the median transplanted dose of CD34+ cells (2.1-13.55). Liver toxicity was observed in 6 (17.6%) patients. Eight patients suffered from pulmonary side effects. The median number of previous chemotherapies was 2 (2-4). In all lymphoma patients, the complete response rate was 61.8% (n = 21), whereas the disease progression rate was 32.3% (n = 11). Transplantation-related mortality on the 100th day was 8.8% (n = 3). Three-year overall survival was 57.17%.CONCLUSION: When the literature was reviewed, the studies with the BuCyE preparation regimen in patients with r/rHL were limited. This conditioning regimen was found to have fewer side effects and a lower cost. It can be preferable when compared to carmustine (BCNU), etoposide, cytarabine (ARA-C), and melphalan (known as BEAM) in r/rHL.Öğe The Experience of Ibrutinib in Chronic Graft-Versus-Host Disease in Patients Undergoing Allogeneic Hematopoietic Stem Cell Transplantation: Single Center Experience(Galenos Publ House, 2023) Sarici, Ahmet; Erkurt, Mehmet Ali; Kuku, Irfan; Kaya, Emin; Berber, Ilhami; Bicim, Soykan; Hidayet, EmineIntroduction: Chronic graft-versus host disease (GVHD) is a serious complication that develops in 35-50% of patients in the late period after allogeneic hematopoetic stem cell transplantation. About half of the patients are resistant to corticosteroids, which is the first-line treatment of chronic GVHD, and therefore new treatment options that can be effective in chronic GVHD are needed. In the present study, we aimed to share our experience with the use of ibrutinib therapy in patients with steroid-resistant chronic GVHD who have previously received multiple lines of systemic therapy. Methods: The characteristics and clinical outcomes of steroid-resistant chronic patients with GVHD receiving ibrutinib were retrospectively reviewed. Results: A total of 10 steroid resi-stant chronic patients with GVHD who received ibrutinib was included. While 50% of the patients had more than one organ involvement, 50% had a single organ involvement. The most commonly affected organs were the skin and liver. The patients received a median of three lines of systemic therapy before ibrutinib. After a median of 210 days of ibrutinib usage, the complete response rate of patients was 40% and the partial response rate was 40%. Corticosteroids were completely discontinued in 30% of patients after ibrutinib were initiated. Before ibrutinib, patients were given a median of 0.3 mg/kg methylprednisolone. The median methylprednisolone dose after ibrutinib was 0.03 mg/kg. Conclusion: Ibrutinib therapy causes a quite high overall response in steroid resistant chronic patients with GVHD and appears to be a good option in these patients.Öğe The frequency of HLA A, B, C, DP, DQ, DR allele in patients of Turkish and Syrian nationals with allogeneic stem cell transplantation(Wolters Kluwer Medknow Publications, 2022) Kaya, Ahmet; Erkurt, Mehmet Ali; Kuku, Irfan; Kaya, Emin; Berber, Ilhami; Bicim, Soykan; Hidayet, EmineBACKGROUND: In allogeneic stem cell transplantation, donor and recipient HLA tissue compatibility is essential for the success of stem cell transplantation. HLA tissue compatibility percentage is the most important parameter that increases the success of transplantation in donor selection. Pretransplant HLA tissue typing can be looked at in low and high resolution according to the facilities of the center and the urgency of transplantation. Many centers evaluate HLA HLA-A, B, C, DP, DQ, DR tissue types before allogeneic bone marrow transplantation. HLA tissue types differ in many races and even between unrelated individuals of the same race. AIMS: This study aimed to show the common human leukocyte antigen (HLA) rates and differences in Syrian and Turkish ethnicity patients who underwent allogeneic stem cell transplantation in our center. MATERIALS AND METHODS: HLA tissue similarities between Turkish and Syrian patients were revealed by examining the HLA tissue records of Turkish and Syrian patients who applied to the bone marrow transplant unit of Inonu University Turgut Ozal Medical Center between December 2009 and November 2021 for allogeneic stem cell transplantation. RESULTS: As a result of our study, it has been observed that there are similarities in terms of HLA A*02, HLA B*35, HLA C 04,07,12, HLA DP*02,04,11 HLA DQ*02,03,05,06, HLA DR*01,03,11,13 in Turkish and Syrian patients. High resolution HLA subgroups of the patients are shown in Tables three and four. CONCLUSION: In allogeneic stem cell transplantation, there may be similar HLA tissue types among ethnic groups.Öğe Non-Infectious Complications in Patients with Allogeneic Hematopoietic Stem Cell Transplantation- Single Center Experience from Eastern Anatolia(2022) Karaman, Sevtap; Sarıcı, Ahmet; Erkurt, Mehmet Ali; Biçim, Soykan; Hidayet, Emine; Kaya, Ahmet; Kuku, İrfanIntroduction: The aim of this study was to investigate late non-infectious complications in patients with allogeneic hematopoietic stem cell transplantation (HSCT). Materials and Methods: The records of 143 patients who underwent allogeneic HSCT between 01.02.2011 and 31.12.2018 in Inonu University Turgut Özal Medical Center Department of Hematology were retrospectively reviewed. Results: In our study, late non-infectious complications were seen in 68 % of the patients. Graft Versus Host Disease (GVHD) was observed in 43 patients (33%) %. The most common GVHD involvement sites were skin and liver. It was determined that the number of CD 34 (+) cells had a significant effect on the development of chronic GVHD (p=0,01) and late stage complications (p=0,016). Chronic GVHD and late complication rates were found to be lower in the group given high CD 34 (+) cell count. When the patients were grouped according to the preparation regimens, statistically significantly more complications were observed at the rate of 78.8% in the patients in the myeloablative regimen group (p=0.005). It was observed that the rate of recurrence of the primary disease, renal complications and neurological complications was higher in the 90-120th days after transplantation. Ocular and GVHD complications were more likely to develop later. It was observed that endocrine complications were encountered equally in both early and late periods. Discussion: It was seen that, late complications were common in patients who underwent allogeneic HSCT and these complications were mostly related to GVHD and GVHD treatment.Öğe The relationship between bioelectrical impedance parameters and pulmonary artery stiffness in obese subjects(Wiley, 2022) Hidayet, Siho; Bayramoglu, Adil; Hidayet, Emine; Ulutad, Zeynep; Dagtekin, Firat; Guven, Fatih; Karaca, YucelObjectives Obesity is a public health problem that needs to be treated and it occurs as a result of excessive fat accumulation in the body. The relationship between obesity and pulmonary hypertension is well known. The aim of this study is to evaluate the relationship between pulmonary artery stiffness, right ventricular functions and bioelectrical impedance parameters in obese, overweight, and healthy individuals. Methods In this study, 41 obese (17 female and 24 male, mean age 43.5 +/- 10.3), 39 overweight (20 female and 19 male, mean age 38.6 +/- 10.4), 34 healthy control group (19 female and 15 male, mean age 40.5 +/- 8.6) were included. Anthropometric measurements and bioelectrical impedance parameters of all participants were performed. Right ventricular functions and pulmonary artery stiffness were evaluated by using conventional echocardiography. Results Right ventricle myocardial performance index, pulmonary artery stiffness values were statistically different between groups. Positive correlation was observed between pulmonary artery stiffness and Body Mass Index, Waist and Hip circumferences. Significant negative correlation was observed between muscle to fat ratio and pulmonary artery stiffness. In the linear regression analysis, it was observed that the muscle to fat ratio was independent predictor of pulmonary artery stiffness (beta = -1.835; 95%CI(-2.434 - - .784); p < 0.001). Conclusions This study showed that right ventricular function was impaired and pulmonary artery stiffness increased in obese individuals. These findings could be considered as early markers of pulmonary hypertension in obese patients who do not yet have clinical evidence of cardiovascular disease.Öğe Relationship between epicardial adipose tissue and body muscle-to-fat ratio in patients with type 2 diabetes mellitus(2020) Hidayet, Siho; Hidayet, EmineAbstract: Type 2 Diabetes mellitus (T2DM) is shown as an important risk factor for the development of cardiovascular diseases worldwide. Epicardial adipose tissue (EAT) has been associated with cardiovascular diseases. The body muscle-to-fat ratio measured by bioelectric impedance (BIA) is associated with cardiac dysfunction. In this study, we aimed to evaluate the relationship between EAT and body muscle-to-fat ratio in T2DM patients. The 125 consecutive T2DM patients and 46 healty volunteers included in the study. EAT was measured by using transthorasic echocardiograph (TTE). Data were obtained BIA analysis in all patient. The muscle-to-fat ratio measured by BIA and EAT was compared between the two groups. There was a significant difference between the groups in terms of waist circumference (WC), fat mass, fat mass ratio, muscle mass and muscle-to-fat ratio. E (p<0.001), Em wave (p<0.001), E/A ratio (p<0.001) and E/Em ratio (p=0.014) were significantly lower in the DM group compared to controls. Also, EAT thickness was higher in the DM group (5.78 ± 0.7 vs 4.89 ± 0.7 p <0.001). While a positive correlation was observed between EAT and BMI (r = 0.445, p <0.001), WC (r = 0.401, p < 0.001), Age (r = 0.237, p = 0.008) and disease duration (r = 0.199, p = 0.049), EAT and muscle-to-fat ratio were negatively correlated (r = -0.615, p <0.001). Age and muscle-to-fat ratio were found to be independent predictors for EAT (?= 0.037; p<0.001; ?=-1.403, p<0.001 respectively). The muscleto- fat ratio measured by bioelectrical impedance technique in type 2 DM patients may provide a more accurate estimate for EAT, which has been shown to be associated with cardiovascular events.
