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Öğe Acute Lymphoblastic Leukemia in Routine Practice: A Turkish Multicenter Study(Galenos Yayincilik, 2019) Ciftciler, Rafiye; Sevindik, Omur Gokmen; Tekgunduz, Ali Irfan Emre; Erkurt, Mehmet Ali; Vural, Filiz; Turgut, Burhan; Kaynar, LeylagulObjective: Significant developments occurred in the clinical management of acute lymphoblastic leukemia (ALL) in adults in recent decades. However, treatment results are still not satisfactory, especially in routine practice. The objective of this study was to evaluate the general clinical features, treatment details, and outcomes of a large group of patients followed in multiple centers in Turkey with a diagnosis of ALL. Materials and Methods: A retrospective analysis of the data of patients with ALL was made, the patients having been diagnosed and treated between January 2003 and June 2017 by different protocols in the hematology clinics of ten different centers. A total of 288 patients, aged between 17 and 76 years old, were included in the study. In this retrospective multicenter analysis of patients with ALL, classification of patients was performed based on treatment period, Philadelphia chromosome positivity, treatment regimen, and administration of allogeneic hematopoietic stem cell transplantation (allo-HSCT). Results: The majority of cases were B-cell in origin, while 224 patients had B-ALL and 64 of the patients had T-ALL. Median follow-up duration for all patients was 18.2 months (range: 0.03-161 months). Philadelphia chromosome positivity was determined in 49 patients (21.9%), and 54 patients (18.8%) were receiving allo-HSCT. After induction chemotherapy, 219 patients (76.0%) achieved complete remission, 32 patients (11.2%) were evaluated as treatment refractory, and 37 patients (12.8%) were deceased. Median overall survival was 47.7 months (95% confidence interval: 36.1-59.2) and median disease-free survival was 23.4 months (95% confidence interval: 6.7-40.0) for all patients. Conclusion: Multicenter studies are extremely important for defining the specific clinical features of a particular disease. The results of this study will make a significant contribution to the literature as they reflect real-life data providing valuable information about the Turkish ALL patient profile.Öğe Autologous hematopoietic progenitor cell mobilization and collection in adult patients presenting with multiple myeloma and lymphoma: A position-statement from the Turkish Society of Apheresis (TSA)(Pergamon-Elsevier Science Ltd, 2017) Tekgunduz, Emre; Arat, Mutlu; Goker, Hakan; Ozdogu, Hakan; Kaynar, Leylagul; Cagirgan, Seckin; Erkurt, Mehmet AliAutologous hematopoietic cell transplantation (AHCT) is a routinely used procedure in the treatment of adult patients presenting with multiple myeloma (MM), Hodgkin lymphoma (HL) and various subtypes of non-Hodgkin lymphoma (NHL) in upfront and relapsed/refractory settings. Successful hematopoietic progenitor cell mobilization (HPCM) and collection are the rate limiting first steps for application of AHCT. In 2015, alinost 1700 AHCT procedures have been performed for MM, HL and NHL in Turkey. Although there are recently published consensus guidelines addressing critical issues regarding autologous HPCM, there is a tremendous heterogeneity in terms of mobilization strategies of transplant centers across the world. In order to pave the way to a more standardized HPCM approach in Turkey, Turkish Society of Apheresis (TSA) assembled a working group consisting of experts in the field. Here we report the position statement of TSA regarding autologous HPCM mobilization strategies in adult patients presenting with MM and lymphoma. (C) 2017 Elsevier Ltd. All rights reserved.Öğe Clinical management, psychosocial characteristics, and quality of life in patients with homozygous familial hypercholesterolemia undergoing LDL-apheresis in Turkey: Results of a nationwide survey (A-HIT1 registry)(Elsevier Science Inc, 2019) Kayikcioglu, Meral; Kuman-Tuncel, Ozlem; Pirildar, Sebnem; Yilmaz, Mehmet; Kaynar, Leylagul; Aktan, Melih; Durms, Rana BerruBACKGROUND: Homozygous familial hypercholesterolemia (HoFH) is a rare, life-threatening inherited disease leading to early-onset atherosclerosis and associated morbidity. Because of its rarity, longitudinal data on the management of HoFH in the real world are lacking, particularly on the impact the condition has on quality of life (QoL), including the impact of the extracorporeal lipid removal procedure apheresis (LA). METHODS: The A-HIT1 study included 88 patients with HoFH aged >= 12 years receiving regular LA in 19 centers in Turkey. Demographic and disease characteristics data were obtained. For patients aged >= 18 years, additional data on psychosocial status were obtained via the SF-36 score, the Hospital Anxiety and Depression Scale, and a HoFH-specific questionnaire. RESULTS: There was no standardized approach to therapy between centers. Mean (+/-SD) frequency of LA sessions was every 19.9 (+/-14) days, with only 11.6% receiving LA weekly, and 85% of patients were not willing to increase LA frequency. The most common concerns of patients were disease prognosis (31%), and physical, aesthetic, and psychological problems (27.5%, 15.9%, and 11.6%, respectively). Lower age at diagnosis was associated with better QoL, lower anxiety, improved functioning, and greater emotional well-being compared to later diagnosis. CONCLUSIONS: These findings demonstrate that adult patients with HoFH undergoing LA, experience significant impairment of QoL with an increased risk of depression. From patients' point of view, LA is time-consuming, uncomfortable, and difficult to cope with. The speed of diagnosis and referral has a considerable impact on patient well-being. (C) 2019 National Lipid Association. All rights reserved.Öğe Diffuse Large B-Cell Lymphoma in adults aged 65 years and older: A retrospective multicenter analysis of clinical characteristics and therapeutic outcomes(Cig Media Group, Lp, 2015) Dogu, Mehmet Hilmi; Keklik, Muzaffer; Terzi, Hatice; Korkmaz, Serdal; Erkurt, Mehmet Ali; Cagliyan, Gulsum Akgun; Kaynar, Leylagul[Abstract Not Available]Öğe Hematopoietic Stem Cell Transplantation for Patients with Paroxysmal Nocturnal Hemoglobinuria with or without Aplastic Anemia: A Multicenter Turkish Experience(Galenos Yayincilik, 2021) Yilmaz, Fergun; Soyer, Nur; Seval, Guldane Cengiz; Bozdag, Sinem Civriz; Topcuoglu, Pervin; Unal, Ali; Kaynar, LeylagulObjective: Although inhibition of the complement system at different steps is a promising therapy modality in patients with paroxysmal nocturnal hemoglobinuria (PNH), allogeneic hematopoietic stem cell transplantation (HCT) is still the only curative therapy, especially for patients with intractable hemolysis or bone marrow failure. The aim of this study is to evaluate the outcomes of allogeneic HCT in PNH patients with aplastic anemia (PNH-AA) or without. Materials and Methods: Thirty-five PNH/PNH-AA patients who were treated with allogeneic HCT in 10 transplantation centers in Turkey were retrospectively analyzed. Results: Sixteen (45.7%) and 19 (54.3%) patients were diagnosed with classical PNH and PNH-AA, respectively. The median age of the patients was 32 (18-51) years. The 2-year overall survival (OS) rate and rate of graft-versus-host disease-free, failure-free survival (GFFS) was 81.2% and 78.1%, respectively. The 2-year OS in cases of classical PNH and PNH-AA was 81.3% and 79.9%, respectively (p=0.87), and 2-year GFFS in cases of PNH and PNH-AA was 79% and 76% (p=0.977), without statistical significance. The OS and GFFS rates also did not differ between transplantations with matched sibling donors (MSDs) and matched unrelated donors (MUDs). Conclusion: Allogeneic HCT with MSDs or MUDS is a good option for selected patients with classical PNH and PNH-AA. In particular, patients with debilitating and refractory hemolysis and patients with bone marrow failure might form an excellent group of candidates for allogeneic HCT.Öğe Mental status and physical activity in patients with homozygous familial hypercholesterolemia: A subgroup analysis of a nationwide survey (A-HIT1 registry)(Elsevier Science Inc, 2020) Tuncel, Ozlem Kuman; Kayikcioglu, Meral; Pirildar, Sebnem; Yilmaz, Mehmet; Kaynar, Leylagul; Aktan, Melih; Durmus, Rana BerruBACKGROUND: Homozygous familial hypercholesterolemia (HoFH) is a rare, life-threatening disease due to high serum low-density lipoprotein (LDL) cholesterol levels. LDL cholesterol-lowering interventions are fundamental for patients with HoFH. OBJECTIVE: It was aimed to investigate the association between the mental status of patients with HoFH and healthy lifestyle behaviors. METHODS: This subgroup analysis of the A-HIT1 population included the data of patients aged >= 18 years with a clinical diagnosis of HoFH undergoing therapeutic LDL apheresis. Besides the demographic and clinical characteristics of patients, healthy lifestyle behaviors were assessed, and psychiatric symptoms were screened by Symptom Check List (SCL-90-R). RESULTS: The highest percentage for pathology was observed in dimensions of obsessive-compulsive, somatization, interpersonal sensitivity, and depression in SCL-90-R. Patients with any cardiovascular condition have more psychiatric symptoms in different fields of SCL-90-R. The outcomes of the correlative analysis indicated that lower the age of the first coronary event better the psychiatric status, probably denoting a better adaptation to disease and its treatment. Among 68 patients, 36 patients were not exercising regularly. Patients with regular physical activity had significantly lower scores in most dimensions of SCL-90-R and there was no association between regular physical activity and other investigated variables. The strongest predictor of regular exercising was global severity index of SCL-90-R. CONCLUSION: In the HoFH population, there was a high prevalence of mental disturbances. Better psychiatric status was associated with regular exercising. Therefore, assessing the mental status of patients with HoFH and referring patients in need, to a psychiatrist, may improve the outcome of patients. (C) 2020 National Lipid Association. All rights reserved.Öğe A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study(Pergamon-Elsevier Science Ltd, 2018) Tekgunduz, Emre; Yilmaz, Mehmet; Erkurt, Mehmet Ali; Kiki, Ilhami; Kaya, Ali Hakan; Kaynar, Leylagul; Alacacioglu, InciThrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTSI3 activity/antiADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CAHUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1-75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE. (C) 2018 Elsevier Ltd. All rights reserved.Öğe A multicenter experience of thrombotic microangiopathies in turkey: the turkish hematologyresearch and education group (threg)-tma01 study(Pergamon-elsevıer scıence ltd, the boulevard, langford lane, kıdlıngton, oxford ox5 1gb, england, 2018) Tekgunduz, Emre; Yilmaz, Mehmet; Erkurt, Mehmet Ali; Kiki, Ilhami; Kaya, Ali Hakan; Kaynar, LeylagulThrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTSI3 activity/antiADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CAHUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1-75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE. (C) 2018 Elsevier Ltd. All rights reserved.Öğe A nation-wide survey of patients with homozygous familial hypercholesterolemia phenotype undergoing LDL-apheresis in Turkey (A-HIT 1 registry)(Elsevier Ireland Ltd, 2018) Kayikcioglu, Meral; Tokgozoglu, Lale; Yilmaz, Mehmet; Kaynar, Leylagul; Aktan, Melih; Durmus, Rana Berru; Gokce, CumaliBackground and aims: Homozygous familial hypercholesterolemia (HoFH) is a genetic condition characterized by lethally high levels of low-density lipoprotein cholesterol (LDL-C) from birth, and requires rapid and aggressive intervention to prevent death due to coronary heart disease and/or atherosclerosis. Where available, lipoprotein apheresis (LA) is the mainstay of treatment to promote survival. Methods: A-HIT1 registry was conducted with the aim of providing insight to the real-life management of HoFH patients undergoing LA in Turkey, where LA procedures are fully reimbursed and widely available. Participating centers provided patient information, including family history, treatment patterns and relevant laboratory values, via a standard questionnaire. Results: The study evaluated 88 patients (mean age: 27 +/- 11 years, 41 women) in 19 centers. All patients were receiving regular LA with a clinical diagnosis of HoFH. Mean age at first symptom disease was 10 +/- 10 years, and at diagnosis it was 12 +/- 11 years; 74.7% were diagnosed before age 15 years; and only 31% before the age of 7. First referral of most patients was to pediatricians. Early onset coronary artery disease was present in 57.8% of patients. Mean age at first LA was 21 +/- 12 years. Only 11 (12.5%) patients were undergoing LA weekly. Mean frequency of apheresis sessions was 19 +/- 13 days. For the last four LA sessions, LDL-C levels reached the target in only in 5.7% of patients. Conclusions: Diagnosis of HoFH is delayed, and LDL targets are not reached. LA frequencies are not optimal. Urgent attention is needed to support the survival of patients with HoFH. (c) 2018 Elsevier B.V. All rights reserved.Öğe A nation-wide survey of patients with homozygous familial hypercholesterolemia phenotypeundergoing ldl-apheresis in turkey (a-hıt 1 registry)(Elsevıer ıreland ltd, elsevıer house, brookvale plaza, east park shannon, co, clare, 00000, ıreland, 2018) Kayikcioglu, Meral; Tokgozoglu, Lale; Yilmaz, Mehmet; Kaynar, Leylagul; Aktan, Melih; Durmus, Rana Berru; Gokce, CumaliBackground and aims: Homozygous familial hypercholesterolemia (HoFH) is a genetic condition characterized by lethally high levels of low-density lipoprotein cholesterol (LDL-C) from birth, and requires rapid and aggressive intervention to prevent death due to coronary heart disease and/or atherosclerosis. Where available, lipoprotein apheresis (LA) is the mainstay of treatment to promote survival. Methods: A-HIT1 registry was conducted with the aim of providing insight to the real-life management of HoFH patients undergoing LA in Turkey, where LA procedures are fully reimbursed and widely available. Participating centers provided patient information, including family history, treatment patterns and relevant laboratory values, via a standard questionnaire. Results: The study evaluated 88 patients (mean age: 27 +/- 11 years, 41 women) in 19 centers. All patients were receiving regular LA with a clinical diagnosis of HoFH. Mean age at first symptom disease was 10 +/- 10 years, and at diagnosis it was 12 +/- 11 years; 74.7% were diagnosed before age 15 years; and only 31% before the age of 7. First referral of most patients was to pediatricians. Early onset coronary artery disease was present in 57.8% of patients. Mean age at first LA was 21 +/- 12 years. Only 11 (12.5%) patients were undergoing LA weekly. Mean frequency of apheresis sessions was 19 +/- 13 days. For the last four LA sessions, LDL-C levels reached the target in only in 5.7% of patients. Conclusions: Diagnosis of HoFH is delayed, and LDL targets are not reached. LA frequencies are not optimal. Urgent attention is needed to support the survival of patients with HoFH. (c) 2018 Elsevier B.V. All rights reserved.Öğe Retrospective evaluation of patients with primary mediastinal large B-Cell lymphoma: Real life experience(2021) Guven, Zeynep Tugba; Celik, Serhat; Kaynar, Leylagul; Keklik, Muzaffer; Eser, Bulent; Cetin, Mustafa; Unal, AliAim: Primary mediastinal large B-cell lymphoma (PMBCL) is a type of lymphoma that forms approximately 3 % of non-Hodgkin lymphomas that often encounter with mass. The aim of this study was to present the epidemiological characteristics, response rates of the treatment and the survival of PMBCL patients in our single center. Materials and Methods: Patient demographics, treatment regimens, survival rates of PMBCL patients were retrospectively analyzed. Results: There are 15 patients in our study. Most of the patients were female (n:9, 60%). The median age at the time of diagnosis was 35.4. Nine patients applied with a bulky lesion in the mediastinum. Most of the patients have been treated with DA-EPOCH-R (dose-adjusted etoposide, doxorubicin, cyclophosphamide, vincristine and prednisone with rituximab) (n:13, 87%) and 2 (n:2, 13%) patients have been treated with R-CHOP (doxorubicin, cyclophosphamide, vincristine and prednisone with rituximab) regimens for 6 cycles. Eight patients (53.3%) received involved-field radiotherapy on the mediastinum. After the first-row chemotherapy, total remission rate was 93.3%. Thirteen (87%) of the patients were still in remission and alive. The median follow-up time in our study was 43 months (13 -81). Mean disease-free survival was 67.43 months and overall survival was 72.87 months. The overall and disease-free survival rate was 86.7 % and 80%. Conclusions: In our study, most patients responded to the treatment and are still being followed in remission.Öğe Therapeutic plasma exchange in patients with neurologic diseases: Retrospective multicenter study(Pergamon-Elsevier Science Ltd, 2008) Kaynar, Leylagul; Altuntas, Fevzi; Aydogdu, Ismet; Turgut, Burhan; Kocyigit, Ismail; Hacioglu, Sibel Kabukcu; Ismailogullari, SevdaTherapeutic plasma exchange (TPE) is commonly used in many neurological disorders where an immune etiology was known or suspected. We report our experience with TPE performed for neuroimmunologic disorders at four university hospitals. The study was a retrospective review of the medical records of neurological patients (n = 57) consecutively treated with TPE between April 2006 and May 2007. TPE indications in neurological diseases included Guillain-Barre Syndrome (GBS) (n = 41), myasthenia gravis (MG) (n = 11), acute disseminated encephalomyelitis (ADEM) (n = 3), chronic inflammatory demyelinating polyneuropathy (CIDP) (n = 1) and multiple sclerosis (MS) (n 1). Patient median age was 49; there was a predominance of males. Twenty-two patients had a history of other therapy including intravenous immunoglobulin (IVIG), steroid, azothioprin, and pridostigmine prior to TPE. Another 35 patients had not received any treatment prior to TPE. All patients were classified according to the Hughes functional grading scores pre- and first day post-TPE for early clinical evaluation of patients. The TPE was carried out 1-1.5 times at the predicted plasma volume every other day. Two hundred and ninety-four procedures were performed on 57 patients. The median number of TPE sessions per patient was five, and the median processed plasma volume was 3075 mL for each cycle. Although the pre-TPE median Hughes score of all patients was 4, it had decreased to grade I after TPE. While the pre-TPE median Hughes score for GBS and MG patients was 4, post-TPE scores were decreased to grade 1. Additionally, there was a statistically significant difference between post-TPE Hughes score for GBS patients with TPE as front line therapy and patients receiving IVIG as front line therapy (1 vs. 3.5; p = 0.034). Although there was no post-TPE improvement in Hughes scores in patients with ADEM and CIDP, patients with MS had an improved Hughes score from 4 to 1. Mild and manageable complications such as hypotension and hypocalcemia were also observed. TPE may be preferable for controlling symptoms of neuroimmunological disorders in early stage of the disease, especially with GBS. (C) 2008 Elsevier Ltd. All rights reserved.
