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Background and aim: Uric acid elevation has been shown to be an important risk factor for cardiovascular and cerebrobascular disease. QT dispersion (QTd) is a parameter that shows the heterogeneity of ventricular repolarization and can be calculated noninvasively from surface electrocardiography. Increased QTd has been associated with severe arrhythmia and risk of sudden death in many patients and disease groups. In this context, we aimed to investigate the effect of uric acid levels on QTd and the effects of decrease in uric acid levels on QTd.

Objective: The aim was to evaluate patients with pesticide poisoning admitted to the intensive care unit in Ataturk Research and Training Hospital in Izmir, Turkey's third largest city, between 2005 2006. Material and method: There were 23 cases admitted to Ataturk Research and Training Hospital during the 2005 2006 period and all were diagnosed with the same descriptive qualities pesticide poisoning. All cases were taken into consideration. Information regarding the patients was obtained from their registration and case files. The research included the patient's age, sex, residential area, work, social security status, how they were exposed and why, length of stay in the hospital, clinic situation, resulting symptoms, laboratory findings such as acetylcholinesterase level, ALT (alanine aminotranferease), AST (aspartate aminotransferase), INR (International Normalized Ratio) levels and results. The evaluations for both average and standard deviations were taken and the descriptive characteristics according to percentage value were done with the datum SPSS 11.0 program. Results: In 2005 12 cases, in 2006 11 cases, a total of 23 incidents of pesticide poisoning were admitted to the Ataturk Research and Training Hospital. The average age of the 23 cases was 31.3±11.9 years (15-68), the median value 29.0. The findings showed that 87.0% were 20 years or over, of the total cases 56.5% were women, 43.5% were men, 65.2% were farmers, 21.7% were unemployed, 8.7% were students and 4.3% were workers. Living areas were, 21.7% within the centre of Izmir, 78.3% within countryside. 59.8 % had no social security. 47.8% of the cases were admitted to the hospital during the summer months, 91.3% took the active material orally, 87.0% for suicidal reasons. 73.9% of the cases remained in the hospital for 5 days or more, 91.6% of the cases were admitted to the hospital between 17.00 and 8.00 hours. Of the patients 52.2% had acetylcholinesterase level low (3,999 µkat/l or lower), 21.7% had ALT level 35 µkat/l or over, 17.4% had AST level 35 µkat/l or over, and 52.2% had INR level 1.2 or under. Of the 23 cases 13.0% (3 cases) died. When the patients presented themselves to the hospital the symptoms most observed were an increase of secretion 91.3%, nausea 82.6%, bradycardia 78.3%, tachycardia 21.7%, perspiration 73.3%, myosis 78.3%, headache 78.3%, hypotension 73.9%, confusion 56.5%, vomiting 43.5%, respiratory failure 43.5%, tremors 39.1%, stomach pains 34.8%, diarrhea 13.0%, melaena 8.7% and haematuria 4.3%. Discussion and conclusions: There is insufficient control on the sale of pesticides in Turkey. Especially during spring and summer those residing in the countryside are under a higher risk. Consistent with other reports organophosphorus compound, a frequently used pesticide, was the most common cause for poisoning in this series. Almost all of the patients were young adults and motive of poisoning was mostly intentional reflecting suicidal attempt in the face of stress. [ABSTRACT FROM AUTHOR]

Objective: In this study, we aimed to obtain real-life data on the use of antimyeloma agents, which significantly increase overall survival (OS) in multiple myeloma (MM) patients, in primary plasma cell leukemia (pPCL) patients with a poor prognosis., Materials and Methods: Data from 53 patients who were diagnosed with pPCL between 2011-2020 and who used at least one proteasome inhibitor (PI) and/or immunomodulatory (IMID) agent were analyzed retrospectively. Depending on the year of pPCL diagnosis, 20% leukocytes or ≥2×109/L plasma cells in the peripheral blood were used., Results: The median age of the patients was 58 years, and 23 (43.4%) patients were over 65 years of age. For first-line treatment, PI or IMID alone was used in 31 (58.5%) patients, and PI and IMID were used simultaneously in 15 (28.3%) patients. Additionally, 21 (39.6%) patients received transplantation, and 13 (24.5%) patients received maintenance treatment. The median progression-free survival was 4 (1-42) months. When patients whose primary disease was refractory to first-line therapy were excluded, the duration of treatment was 6.5 months. The median OS was 15 months, with a median follow-up of 15 months. Only 7 (13.2%) of the patients were alive at the last follow-up visit. Those with higher beta-2 microglobulin levels and ISS stage 3 and nontransplant patients receiving first-line treatment had shorter OS (p=0.005, p=0.02 and p=0.008, respectively). Otherwise, the concomitant use of PIs and IMIDs, the addition of chemotherapy to induction therapy, and the response to induction therapy or maintenance therapy did not affect OS., Conclusion: In our study, as in previous similar studies, we could not see the increased survival trend in pPCL which is observed in MM. New studies are needed for pPCL patients, which is likely to increase with the new diagnostic criteria, based on current agents and information in MM.

Evans syndrome (ES) is a spectrum of diseases in which the combination of autoimmune hemolytic anemia and immune thrombocytopenia or sometimes neutropenia. ES has been accepted usually as an idiopathic condition, but it may be secondary. The coexistence of autoimmune cytopenias and Hodgkin lymphoma (HL) is rarely observed and the rate of ES in HL patients is not clear. Here we describe a 56-year-old male patient who presented with ES and was diagnosed with HL. After corticosteroids, intravenous immunoglobulin (IVIG) and ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) treatment, immune cytopenias were completely resolved. The literature is also reviewed and we found 16 cases in which HL and ES coexist. Although AIHA and immune thrombocytopenia usually develop simultaneously, they rarely occur at different times. Many aspects of the pathogenesis are unknown, but it is thought to be a complex immunological background. Corticosteroids and/or IVIG are the most commonly used first-choice drugs in the initial treatment of ES. Response rates to treatment are variable and response to treatment may be poor, particularly with underlying conditions. If detected, the underlying lymphoma should be treated., Competing Interests: No conflict of interest was declared by the authors.

Competing Interests: Thomas Chatzikonstantinou received honoraria from AbbVie. Lydia Scarfò received honoraria from AbbVie, AstraZeneca, BeiGene, Lilly, Janssen, Octapharma. Gloria Iacoboni received honoraria and travel support from Novartis, Kite/Gilead, Bristol‐Myers Squibb, Abbvie, Autolus, Miltenyi, and AstraZeneca. Rosa Collado received support for attending meetings from Janssen‐Cilag and S.A. Sara Galimberti received honoraria support for attending meetings from AbbVie, AstraZeneca, Jazz, Novartis, and Incyte, honoraria from Roche, Celgene, Pfizer, and Janssen, and support for attending meetings from Jazz, AstraZeneca, and Roche. Romain Guieze received honoraria, consulting fees, and support for attending meetings from AbbVie, Beigene, Roche, Janssen, and AstraZeneca. Eleftheria Hatzimichael received honoraria from AbbVie, Janssen‐Cilag, AstraZeneca, and Roche. Yair Herishanu received honoraria from Janssen, AbbVie, Roche, AstraZeneca, Medion, and Lilly. José‐Ángel Hernández‐Rivas received honoraria as a consultant from Janssen, AbbVie, AstraZeneca, Lilly, and BeiGene and support for attending meetings from Janssen, AbbVie, AstraZeneca, and BeiGene. Ozren Jaksic received honoraria from Johnson and Johnson, AstraZeneca, and Lilly, honoraria from Johnson and Johnson, AbbVie, AstraZeneca, and Lilly, and support for attending meetings from Johnson and Johnson, and AbbVie. Kamel Laribi received consulting fees from AbbVie, AstraZeneca, Janssen, Beigene, Takeda, and Novartis. Maya Koren‐Michowitz received honoraria from Novartis, Pfizer, and Gad Medical LTD. and support for attending meetings from Novartis. Arnon P. Kater received advisory board fees and research money from Janssen, AbbVie, BMS, AstraZeneca, and Roche/Genentech, and support for attending meetings from Janssen and AbbVie. Ioannis Kotsianidis received honoraria and consulting fees from AbbVie and Janssen. Ivana Milosevic received honoraria from AbbVie, Roche, Sandoz, AstraZeneca, and Janssen, and support for attending meetings from AbbVie, Roche, and Takeda. Almudena Navarro‐Bailón received honoraria, advisory board fees, and support for attending meetings from AbbVie, AstraZeneca, Takeda, Janssen, and Beigene. Jacopo Olivieri received honoraria from AbbVie, AstraZeneca, and Janssen. Gianluigi Reda received consulting fees from AbbVie, AstraZeneca, Janssen, and Beigene, and is currently employed by AstraZeneca. Gian M. Rigolin received honoraria for participation in speaker's bureau from AbbVie, Astra Zeneca, Beigene, and Janssen, and support for attending meetings from Janssen. Mattia Schipani received honoraria and support for attending meetings from AstraZeneca, AbbVie, and Janssen‐Cilag and owns shares of stock in AbbVie, AstraZeneca, Merck, Eli Lilly, Sanofi, Johnson and Johnson, Pfizer, Gilead, and GSK. Tereza Shokralla and Stephan Stilgenbauer reports research funding from, consultancy or advisory role for, honoraria from, speakers' bureau participation for, and travel support from AbbVie, Amgen, AstraZeneca, BeiGene, Bristol Myers Squibb, Celgene, Gilead, GlaxoSmithKline, Hoffmann‐La Roche, Incyte, Infinity, Janssen, Novartis, and Sunesis. Eric Tse received support for attending meetings from Takeda. Theodoros Vassilakopoulos received honoraria from Takeda, Roche, Genesis Pharma, Merck, Novartis, Gilead, Sandoz, AstraZeneca, Integris, and Servier, and support for attending meetings from Takeda, Roche, Genesis Pharma, Merck, Pfizer, and Winmedica. Candida Vitale received honoraria from AbbVie, consulting fees from AstraZeneca, and support for attending meetings from AstraZeneca, Takeda, and Janssen. Renata Walewska received honoraria from AbbVie, AstraZeneca, and Beigene, support for attending meetings from Janssen, AbbVie, and AstraZeneca, and advisory board fees from AbbVie, AstraZeneca, Janssen, Beigene, and SecuraBio. Lucrecia Yañez received honoraria from AbbVie, AstraZeneca, Novartis, Gilead, Janssen, Jazz, MSD, and Pfizer, support for attending meetings from AbbVie, AstraZeneca, Gilead, Janssen, and Pfizer, and advisory board fees from AbbVie, AstraZeneca, Jazz, Janssen, Beigene, and Celgene. Francesc Bosch received consulting fees, honoraria, and payment for expert testimony from AbbVie, Genentech, Novartis, Takeda, Janssen, Roche, Mundipharma, Celgene/BMS, AstraZeneca, Lilly, Beigene, Gilead and TG Therapeutics, Advantage Allogene, Lava Therapeutics, and Enterome. Stephen Mulligan received advisory board fees from AbbVie, AstraZeneca, Janssen, Roche, and BeiGene. Maria Angelopoulou received consulting fees from AbbVie, Takeda, Janssen, Roche, Genesis, Gilead, and Amgen and honoraria from AbbVie, Takeda, Roche, Genesis, Gilead, and Novartis. Fatih Demirkan received support for attending meetings from Janssen and AbbVie. Tomas Papajík received honoraria and advisory board fees from AbbVie, Janssen‐Cilag, and AstraZeneca, and support for attending meetings from AstraZeneca. Marta Coscia received honoraria, advisory board fees, and support for attending meetings from AbbVie, AstraZeneca, and Janssen. Constantine Tam received honoraria from AbbVie, Beigene, Janssen, and LOXO. Antonio Cuneo received honoraria, advisory board fees, and support for attending meetings from AbbVie, AstraZeneca, Beigene, Janssen, and Lilly. Gianluca Gaidano received honoraria from Abbvie, AstraZeneca, BeiGene, Hikma, Incyte, Janssen, and Lilly. Niki Stavroyianni received honoraria from Janssen, AbbVie, AstraZeneca, and Lilly, and support for attending meetings from Janssen and AstraZeneca. Lukas Smolej received consulting fees, honoraria, and support for attending meetings from AbbVie, AstraZeneca, and Janssen and advisory board fees from AbbVie and AstraZeneca. Martin Spacek received honoraria and consulting and advisory board fees, and support for attending meetings from AbbVie, AstraZeneca, and Janssen. Michael Doubek received research support and honoraria from AbbVie, AstraZeneca, and Janssen. Eugene Nikitin received honoraria from AbbVie. Kostas Stamatopoulos received research support from AbbVie, AstraZeneca, Janssen, Novartis, and Roche; honoraria from AbbVie, AstraZeneca, Bristol Myers Squibb, Lilly, and Janssen. Paolo Ghia received research support from AbbVie, AstraZeneca, BMS, Janssen and honoraria from AbbVie, AstraZeneca, BeiGene, BMS, Galapagos, Genmab, Janssen, Loxo Oncology @Lilly, MSD, Roche, and is an Editor of HemaSphere. Georgios Karakatsoulis, Eva Minga, Dimitra Chamou, Jana Kotaskova, Christos Demosthenous, Elisa Albi, Miguel Alcoceba, Salem Al‐Shemari, Thérèse Aurran‐Schleinitz, Francesca Bacchiarri, Sofia Chatzileontiadou, Zadie Davis, Marcos Daniel de Deus Santos, Maria Dimou, Elena Dmitrieva, David Donaldson, Gimena Dos Santos, Barbara Dreta, Maria Efstathopoulou, Shaimaa El‐Ashwah, Alicia Enrico, Andrzej Frygier, Andrea Galitzia, Eva Gimeno, Valerio Guarente, Sean Harrop, Elżbieta Kalicińska, Volkan Karakus, Bonnie Kho, Maria Kislova, Εliana Konstantinou, Zuzana Kubova, Jorge Labrador, Deepesh Lad, Luca Laurenti, Thomas Longval, Alberto Lopez‐Garcia, Juan Marquet, Stanislava Maslejova, Carlota Mayor‐Bastida, Biljana Mihaljevic, Fatima Miras, Riccardo Moia, Marta Morawska, Uttam K. Nath, Irina Panovska‐Stavridis, Maria Papaioannou, Cheyenne Pierie, Anna Puiggros, Rosa Ruchlemer, Annett Schiwitza, Yandong Shen, Tereza Shokralla, Martin Simkovic, Svetlana Smirnova, Dina S. A. Soliman, Tamar Tadmor, Kristina Tomic, Andrea Visentin, George Vrachiolias, Vojin Vukovic, Zhenshu Xu, Munci Yagci, Mohamed Yassin, Jana Zuchnicka, David Oscier, Alessandro Gozzetti, Panagiotis Panagiotidis, Blanca Espinet, Paolo Sportoletti, Gerassimos A. Pangalis, Viola M. Popov, Bella Biderman, Roberta Murru, Rainer Claus, Livio Trentin, Darko Antic, Olga B. Kalashnikova, Mark Catherwood, Sarka Pospisilova, and Anastasia Chatzidimitriou have no conflict of interest to disclose.

Predictive prognostic scoring (PS) systems are not primarily applicable to elderly patients with classical Hodgkin lymphoma (cHL). The objective of this study was to develop a PS system for these patients. The derivation cohort (DC) was utilized for model development, consisting of 97 variables. The resulting algorithm was named as Hodgkin's Lymphoma Early Death in the Elderly within 12 months (HEDEL12). Internal and external validation cohorts (IVC and EVC) were employed for validation. A total of 286 patients were evaluated retrospectively. In DC 38 of 178 patients died within the first 12 months and overall survival (OS) at 12-month was 78.6%. Independent predictors of HEDEL12 were female sex, low albumin levels (<3.5 g/dL), and ECOG scores 2-4. According to HEDEL12 scores 0-1, OS at 12- months were 89.8% and 91.0% for IVC and EVC, respectively. The HEDEL12 scoring is useful in predicting the survival of advanced-stage cHL patients.

Objectives: This study investigates the roles of HIF-2α, hepcidin, and ghrelin in iron deficiency anemia (IDA), the most widespread nutritional disorder globally., Material and Methods: Fifty IDA patients (18-50 years, BMI 19-25) and 40 healthy volunteers were studied. Hemoglobin, ferritin, hepcidin, HIF-2α, and ghrelin levels were analyzed., Results: IDA patients showed lower hemoglobin, ferritin, hepcidin, and ghrelin levels than the control group, but HIF-2α levels were similar. Positive correlations were observed in both groups between hepcidin and HIF-2α (p < 0.001), hepcidin and ghrelin (p < 0.001), and HIF-2α and ghrelin (p < 0.001). Hemoglobin was correlated positively with HIF-2α, and ferritin was correlated positively with HIF-2α in the patient group., Conclusion: The study suggests that the low hepcidin levels in IDA patients enhance iron absorption. The lack of significant HIF-2α level differences may be due to the absence of chronic hypoxia in current hemoglobin levels of IDA patients. Moreover, the low ghrelin levels in patients and the correlations between ghrelin, hepcidin, and HIF-2α in both groups indicate their involvement in iron metabolism., (© 2024. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

Introduction: Thrombocytopenia is among the most common complications following hematopoietic stem cell transplantation and is associated with increased mortality and morbidity with no standard treatment yet. In this multicenter and retrospective study, we aim to present our multi-center experience of Eltrombopag treatment in patients with isolated thrombocytopenia following HSCT., Material-Method: A total of 73 patients from 5 centers who underwent autologous or allogeneic stem cell transplantation, had no primary disease relapse, all of whom had neutrophil engraftment, complete chimerism, and who were diagnosed with Prolonged Isolated Thrombocytopenia (PIT) or Secondary Failure Of Platelet Recovery (SFPR) were included in the study. The patients were initiated on Eltrombopag at a dose of 50-150 mg. Complete response was defined as a platelet count >50×10 9 /L for 7 consecutive days with no transfusion support., Results: A total of 50.3% of the patients underwent Autologous and 49.7% Allogeneic Stem Cell Transplantation, 54.8% were diagnosed with PIT, and 45.2% were diagnosed with SFPR, and the treatment with 50-150 mg/day Eltrombopag was initiated on the median day +42. Complete response was achieved in 71.2% of these patients on the median day 23 of the treatment. No significant effects of the initial dose (50-150 mg/day) were detected in the Complete Response in the multivariate analysis on response. An insufficient number of Megakaryocytes in the bone marrow before Eltrombopag treatment was determined as an independent risk factor in determining the response (OR 3.57, 95% CI 1.21-10.55). The overall survival of the patients who did not respond to Eltrombopag was found to be significantly worse than that of patients who responded (p=0.022, HR:2.74, 95% CI 1.12-6.54)., Conclusion: As a result of the present study, Eltrombopag treatment was found to be effective and safe in thrombocytopenia that develops following hematopoietic stem cell transplantation. It was concluded that its use may be more effective in patients with sufficient bone marrow megakaryocytes before the treatment and an initial dose of 50 mg/day may be appropriate in terms of cost, effectiveness, and toxicity. Large-scale randomized and controlled prospective studies are needed to determine the roles of Eltrombopag treatment in patients with post-transplant PIT and SFPR., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Objectives: The aim of this study was to retrospectively evaluate the effect of plasmapheresis treatment concomitant with chemotherapy and the number of sessions on renal improvement and survival in patients with newly diagnosed multiple myeloma (MM) presenting with acute kidney injury (AKI)., Material and Methods: Retrospective analysis was performed on 55 newly diagnosed MM patients who were presented with AKI to the Hematology Clinic of University of the Health Sciences Antalya Training and Research Hospital between 2013 and 2021., Results: The study included 55 patients between 39 and 91 years of age and comprised 22 (40%) women and 33 (60%) men. Forty-eight (87.3%) patients were treated with plasmapheresis and chemotherapy. Based on the median number of plasmapheresis sessions, the patients were grouped as ≤ 3 and > 3. A significant difference was observed in both groups between the mean values of repeated measurements at the time of diagnosis, after completion of plasmapheresis treatment, and at 1 month of plasmapheresis, when statistics of differences were evaluated for urea, creatinine, estimated glomerular filtration rate (eGFR) (ml/min), total protein, albumin, and globulin (p < 0.05); however, there was no difference between these parameters and the number of plasmapheresis sessions. The 1.16 (0.56-2.38) fold higher risk of ex found in patients with ≤ 3 plasmapheresis sessions compared to those with > 3 was not statistically significant (p > 0.05)., Conclusion: It was observed that plasmapheresis is beneficial in the short term for renal recovery in the treatment of MM with AKI and that > 3 plasmapheresis sessions have no superior effectiveness in renal improvement or survival., (© 2023. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

Competing Interests: Competing interests: The authors declare no conflict of Interest.

The Virtual Opinions poll Independent Centered on CLL patients' Experience (VOICE) evaluated patients' knowledge about chronic lymphocytic leukemia (CLL), their perspectives on diagnosis and treatment, and their unmet needs. Clinicians and patient advocacy group representatives developed and distributed the survey from March through December 2022 in 12 countries, and 377 patients with ≥1 line of previous CLL treatment responded from Europe, Latin America, the United States, Australia, Egypt, and Turkey. A majority of them (90%; 336/374) relied on their physicians for information regarding CLL and treatment. If at high risk, respondents prefer oral medications to intravenous (78%; 232/296), fixed duration treatment over treatment until progression (69%; 185/270), outpatient over inpatient treatments (91%; 257/283). Over three-fourths of respondents (78%; 286/368) wanted to be involved in treatment decisions, but a minority actually participated (44%; 138/313). COVID-19 vaccinations were widely available (97%; 273/281), but one-fifth (19%; 63/331) were unaware that CLL increases vulnerability to infections. Most patients' physicians explained their treatment options (84%; 297/355), and 90% (271/301) understood their treatment. Notably, >10% would continue treatment normally if they experienced cardiac problems or arrhythmias, whereas 23% would consider stopping treatment if they developed skin cancer. Treatment-associated side effects affected 27% to 43% of patients. These results in a global patient population highlight gaps in patients' knowledge of risk groups, their susceptibility to infections including COVID, and the side effects of common treatments. Such knowledge can guide the appropriate targeting of patient education initiatives by clinicians, advocates, and policymakers., (© 2023 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Background: Patients with chronic lymphocytic leukemia (CLL) have a higher risk of developing other malignancies (OMs) compared to the general population. However, the impact of CLL-related risk factors and CLL-directed treatment is still unclear and represents the focus of this work., Methods: We conducted a retrospective international multicenter study to assess the incidence of OMs and detect potential risk factors in 19,705 patients with CLL, small lymphocytic lymphoma, or high-count CLL-like monoclonal B-cell lymphocytosis, diagnosed between 2000 and 2016. Data collection took place between October 2020 and March 2022., Findings: In 129,254 years of follow-up after CLL diagnosis, 3513 OMs were diagnosed (27.2 OMs/1000 person-years). The most common hematological OMs were Richter transformation, myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Non-melanoma skin (NMSC) and prostate cancers were the most common solid tumors (STs).The only predictor for MDS and AML development was treatment with fludarabine and cyclophosphamide with/without rituximab (FC ± R) (OR = 3.7; 95% CI = 2.79-4.91; p < 0.001). STs were more frequent in males and patients with unmutated immunoglobulin heavy variable genes (OR = 1.77; 95% CI = 1.49-2.11; p < 0.001/OR = 1.89; 95% CI = 1.6-2.24; p < 0.001).CLL-directed treatment was associated with non-melanoma skin and prostate cancers (OR = 1.8; 95% CI = 1.36-2.41; p < 0.001/OR = 2.11; 95% CI = 1.12-3.97; p = 0.021). In contrast, breast cancers were more frequent in untreated patients (OR = 0.17; 95% CI = 0.08-0.33; p < 0.001).Patients with CLL and an OM had inferior overall survival (OS) than those without. AML and MDS conferred the worst OS (p < 0.001)., Interpretation: OMs in CLL impact on OS. Treatment for CLL increased the risk for AML/MDS, prostate cancer, and NMSC. FCR was associated with increased risk for AML/MDS., Funding: AbbVie, and EU/EFPIAInnovative Medicines Initiative Joint Undertaking HARMONY grant n° 116026., Competing Interests: TC received honoraria from AbbVie. LS received consulting fees from AbbVie, BeiGene, AstraZeneca, Lilly, and Janssen, honoraria from Janssen and Octapharma, support for attending meetings from BeiGene and Janssen and advisory board fees from Merck. SMu received advisory board fees from AbbVie, AstraZeneca, Janssen, Roche and BeiGene. JAHR received honoraria as a consultant from Janssen, AbbVie, AstraZeneca, Lilly, and BeiGene and support for attending meetings from Janssen, AbbVie, AstraZeneca, and BeiGene. LI received honoraria from AbbVie, Roche, and Janssen-Cilag. APK received advisory board fees and research money from Janssen, AbbVie, BMS, AstraZeneca, Roche/Genentech, support for attending meetings from Janssen and AbbVie. M-DL received travel expenses from Janssen and AbbVie. GMR received honoraria for participation to speaker's bureau from AbbVie, Astra Zeneca, Beigene, and Janssen, and support for attending meetings from Janssen. JVT received consulting fees from AbbVie, AstraZeneca, BeiGene, and Janssen, honoraria for scientific talks from AbbVie, AstraZeneca, BeiGene, Janssen, Lilly, and Roche, travel support from AbbVie, AstraZeneca, BeiGene, Janssen, Roche, and Lilly, and advisory boards fees for AbbVie, Amgen, AstraZeneca, BeiGene. GI received honoraria from Novartis, BMS, Sandoz, AstraZeneca, Janssen, Kite/Gilead, and Miltenyi, support for attending meetings from Kite/Gilead, AstraZeneca, and AbbVie and advisory board fees from Kite/Gilead, Novartis, BMS, and Autolus. FBi received support for attending meetings from AbbVie. RCo received support for attending meetings from Janssen-Cilag and S.A. SG received honoraria support for attending meetings from AbbVie, AstraZeneca, Jazz, Novartis, and Incyte, honoraria from Roche, Celgene, Pfizer, and Janssen, and support for attending meetings from Jazz, AstraZeneca, and Roche. RGS received support for attending meetings from AbbVie and S.L.U. RG received honoraria, consulting fees, and support for attending meetings from AbbVie, Beigene, Roche, Janssen, AstraZeneca. YH received honoraria from Janssen, AbbVie, Roche, AstraZeneca, Medion, and Lilly. OJ received honoraria from Johnson and Johnson, AstraZeneca, and Lilly, honoraria from Johnson and Johnson, AbbVie, AstraZeneca, and Lilly, and support for attending meetings from Johnson and Johnson, and AbbVie. LK received consulting fees from AbbVie, AstraZeneca, Janssen, Beigene, Takeda, and Novartis. MKM received honoraria from Novartis, Pfizer, and Gad Medical LTD and support for attending meetings from Novartis. IKo received honoraria and consulting fees from AbbVie and Janssen. IM received honoraria from AbbVie, Roche, Sandoz, AstraZeneca, and Janssen, and support for attending meetings from AbbVie, Roche, and Takeda. ANB received honoraria, advisory board fees and support for attending meetings from AbbVie, AstraZeneca, Takeda, Janssen, and Beigene. JO received honoraria from AbbVie, AstraZeneca, and Janssen. GR received consulting fees from AbbVie, AstraZeneca, Janssen, and Beigene, and is currently employed by AstraZeneca. TP received honoraria and advisory board fees from AbbVie, Janssen-Cilag, and AstraZeneca and support for attending meeting from AstraZeneca. MS received honoraria and support for attending meeting from AstraZeneca, AbbVie, and Janssen-Cilag and owns shares of stock in AbbVie, AstraZeneca, Merck, Eli Lilly, Sanofi, Johnson and Johnson, Pfizer, Gilead, and GSK. MSp received honoraria and consulting and advisory board fees, and support for attending meeting from AbbVie, AstraZeneca, and Janssen. ET received support for attending meetings from Takeda. TV received honoraria from Takeda, Roche, Genesis Pharma, Merck, Novartis, Gilead, Sandoz, AstraZeneca, Integris, and Servier and support for attending meetings from Takeda, Roche, Genesis Pharma, Merck, Pfizer, and Winmedica. CV received honoraria from AbbVie, consulting fees from AstraZeneca and support for attending meeting from AstraZeneca, Takeda, and Janssen. RW received honoraria from AbbVie, AstraZeneca, and Beigene, support for attending meetings from Janssen, AbbVie, and AstraZeneca and advisory board fees from AbbVie, AstraZeneca, Janssen, Beigene, and SecuraBio. EWS received honoraria from AbbVie, Roche, and Janssen-Cilag and support for attending meetings from AbbVie. LY received honoraria from AbbVie, AstraZeneca, Novartis, Gilead, Janssen, Jazz, MSD, and Pfizer, support for attending meetings from AbbVie, AstraZeneca, Gilead, Janssen, and Pfizer, and advisory board fees from AbbVie, AstraZeneca, Jazz, Janssen, Beigene, and Celgene. MAn received consulting fees from AbbVie, Takeda, Janssen, Roche, Genesis, Gilead, and Amgen and honoraria from AbbVie, Takeda, Roche, Genesis, Gilead, and Novartis. MC received honoraria, advisory board fees, and support for attending meetings from AbbVie, AstraZeneca, and Janssen. ACu received honoraria, advisory board fees, and support for attending meetings from AbbVie, AstraZeneca, Beigene, Janssen, and Lilly. FD support for attending meetings from Janssen and AbbVie. GG received honoraria, and advisory board fees from AbbVie, AstraZeneca, Beigene, Janssen and advisory board fees from Lilly. EN received honoraria from AbbVie. LSm received consulting fees, honoraria and support for attending meetings from AbbVie, AstraZeneca, and Janssen and advisory board fees from AbbVie and AstraZeneca. NS received honoraria from Janssen, AbbVie, AstraZeneca, and Lilly, and support for attending meetings from Janssen and AstraZeneca. CT received honoraria from AbbVie, Beigene, Janssen, and LOXO. FB received consulting fees, honoraria and payment for expert testimony from AbbVie, Genentech, Novartis, Takeda, Janssen, Roche, Mundipharma, Celgene/BMS, AstraZeneca, Lilly, Beigene, Gilead and TG Therapeutics, Advantage Allogene, Lava Therapeutics, and Enterome. MDo received honoraria and advisory board fees from AbbVie, AstraZeneca and Janssen, advisory board fees from Swixx, and support for attending meetings from Janssen. PG received honoraria and consulting fees from AbbVie, AstraZeneca, BMS, Janssen, Lilly/Loxo Oncology, MSD, and Roche; grant support from AbbVie, AstraZeneca, BMS, Janssen. KS received honoraria from Janssen, AbbVie, Lilly and AstraZeneca, consulting fees and support for attending meetings from Janssen and AstraZeneca. GK, EM, DC, JK, CD, MA, SA, TAS, FBa, MB, ACa, AM, AKM, RC, SC, ACh, ZD, MDi, DD, GDS, BD, ME, SEA, AE, AF, AG, EG, IGGM, AGo, AjG, RGu, SH, EH, SJ, EKa, VK, BK, MK, EK, RJK, JL, DL, IL, TL, ALG, JM, LMR, MMa, SM, CMB, BM, FM, RM, MMo, RMu, UKN, ACO, DO, IPS, MP, ZK, PP, CP, AP, LR, RR, MDDS, AS, YS, MSi, SS, DSAS, TT, KT, AV, GV, VV, ZX, MYa, MY, JZ, DA, BB, MCa, RCl, BE, OBK, LL, GP, PPa, VMP, SP, PS, LT, AC, have no conflict of interest to disclose., (© 2023 Published by Elsevier Ltd.)

Glofitamab is a CD3xCD20 bi-specific antibody with two fragments directed to the CD20 antigen and a single CD3-binding fragment. Encouraging response and survival rates were recently reported in a pivotal phase II expansion trial conducted in patients with relapsed/refractory (R/R) B-cell lymphoma. However, the real-world data of patients of all ages with no strict selection criteria are still lacking. Herein, this retrospective study aimed to evaluate the outcomes of diffuse large B-cell lymphoma (DLBCL) patients who received glofitamab via compassionate use in Turkey. Forty-three patients from 20 centers who received at least one dose of the treatment were included in this study. The median age was 54 years. The median number of previous therapies was 4, and 23 patients were refractory to first-line treatment. Twenty patients had previously undergone autologous stem cell transplantation. The median follow-up time was 5.7 months. In efficacy-evaluable patients, 21% and 16% of them achieved complete response and partial response, respectively. The median response duration was 6.3 months. The median progression-free survival (PFS) and overall survival (OS) was 3.3 and 8.8 months, respectively. None of the treatment-responsive patients progressed during the study period, and their estimated 1-year PFS and OS rate was 83%. The most frequently reported toxicity was hematological toxicity. Sixteen patients survived, while 27 died at the time of the analysis. The most common cause of death was disease progression. One patient died of cytokine release syndrome during the first cycle after receiving the first dose of glofitamab. Meanwhile, two patients died due to glofitamab-related febrile neutropenia. This is the largest real-world study on the effectiveness and toxicity of glofitamab treatment in R/R DLBCL patients. The median OS of 9 months seems promising in this heavily pretreated group. The toxicity related mortality rates were the primary concerns in this study., (© 2023 The Authors. Hematological Oncology published by John Wiley & Sons Ltd.)

Aim: This study aimed to examine the color discrimination ability of patients with transfusion-dependent beta-thalassemia (TDβ-T) in detail using the Farnsworth Munsell (FM) 100-hue test and to evaluate structural changes by swept source-optical coherence tomography (SS-OCT)., Material and Methods: This prospective, sectional study included 40 patients (79 eyes) with TDβ-T and 21 controls (42 eyes). The volunteers underwent a detailed ophthalmological examination and SS-OCT (DRI-OCT, Triton) imaging. Excluded were those with congenital color vision defects detected with the Ishihara pseudoisochromatic test. The patients' color vision was examined using the FM 100-hue test. The total error score (TES), the blue-yellow local error score (b-y LES), and the red-green local error score (r-g LES) were calculated. p <0.05 was considered significant., Results: The mean age was 30.34±6.94 years in the patient group and 32.26±6.43 years in the control group (p = 0.078). The patient group had a significantly lower hemoglobin level (9.25±0.87 g/dL vs. 14±1.79 g/dL, p <0.001) and a significantly higher ferritin level (2665.56±2658.05 μg/L vs. 52.87±69.59 μg/L, p<0.001) compared to the control group. The mean TES, b-y LES, and r-g LES were higher in the patients than in the controls (64.84±30.18 vs. 28.45±16.55, p<0.001, 34.21±17.54 vs. 15.67±10.07, p <0.001, and 29.32±15.72 vs. 12.12±7.94, p<0.001, respectively). The patients had a higher b-y LES than r-g LES (34.21±17.54 vs. 29.32±15.72, p = 0.015). Choroidal thickness was lower in the patients than in the controls (284.34±63.55 µm vs. 324.98±88.05 µm, p = 0.043)., Conclusion: We found that the color discrimination ability of the patients with TDβ-T was reduced in both the r-g and b-y color axes compared to the controls, and their color discrimination ability in the b-y color axis was more affected than in the r-g axis., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Ocular involvement is common in transfusion-dependent beta-thalassemia (TDβ-T) patients. We aimed to investigate the effect of splenectomy on optical coherence tomography angiography (OCTA) findings in TDβ-T patients., Methods: The study is a prospective cross-sectional study. A total of 45 eyes of 23 patients with splenectomy (34.04±8.83 years), 18 eyes of 9 patients without splenectomy (27.44±5.43 years), and 54 eyes of 27 controls (33.22±6.44 years) were included. Vessel density in superficial capillary plexus, deep capillary plexus and radial peripapillary capillary, foveal avascular zone, choriocapillaris flow area, choroidal and retinal thickness detected by OCTA were evaluated. p < 0.05 was considered significant., Results: Vessel density of superficial capillary plexus and deep capillary plexus were similar in patients with and without splenectomy, and controls. Choriocapillaris flow area was significantly decreased in patients with splenectomy than that in those without splenectomy and controls (2.02±0.12 vs. 2.17±0.1 and 2.14±0.12; p < 0.001). Choroidal thickness was significantly lower in patients without splenectomy than in patients with splenectomy and controls (260.05±61.02 vs. 305.11±42.13 and 298.89±29.14, p = 0.008). Parafoveal and perifoveal thickness of the full retina and outer retina were significantly lower in patients without splenectomy than in patients with splenectomy and controls (301.06±10.0, 279.78±10.28 vs. 311.04±14.89, 290.87±13.67 and 316.63±13.57, 289.56±9.31, p < 0.001 and p = 0.002; 174.72±7.81, 167.17±6.21 vs. 182.87±8.81, 173.60±7.09 and 185.11±9.26, 173.96±6.79, p = 0.001 and p < 0.001, respectively)., Conclusions: OCTA findings can provide information about the microvascular effects of splenectomy on the retina of patients with TDβ-T., Competing Interests: Declarations of Competing Interest None, (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objective: Acute myeloid leukemia (AML) is a hematological malignancy that frequently affects elderly population. With introducing the hypomethylating agents (HMAs) in elderly AML treatment, survival rates and quality of life have improved. However, long-term management in elderly and frail patients is still a challenge. In the present study, we aimed to determine whether HMA maintenance therapy is required until disease progression in frail and elderly AML patients by examining with a real-life data., Methods: In a multicenter study, we analyzed non-promyelocytic elderly AML patients who were treated with first-line azacitidine or decitabine monotherapy in two different groups, retrospectively. While patients were treated with HMA until progression in the maintenance group, 6+3 cycles of azacitidine or decitabine were administered as a standard care of elderly AML patients in the non-maintenance group. Survival outcomes were compared between the groups., Results: HMA therapy was maintained until progression in 20 patients, and HMA therapy was terminated after 6+3 cycles in 21 patients. Patients received a median of 6 (1-14) HMA cycles during follow-up time. The median 7.5 months of overall survival were observed (2-17 months) in maintenance and 3 months (1-13 months) in non-maintenance groups (p=0.001)., Conclusion: Despite long-term exposure to HMA may appear as a risk factor for complications and toxicities in elderly and frail AML patients, the maintenance of therapy until disease progression provides a significant survival advantage. Therefore, we suggest that HMA therapy should continue until disease progression regardless the sort of HMA., Competing Interests: No conflict of interest was declared by the authors., (© Copyright 2022 by Istanbul Provincial Directorate of Health.)

Thrombotic microanjiopathy (TMA) is a pathological diagnosis characterized by abnormalities of small vessels leading to microvascular thrombosis of arterioles and capillaries. The current prospective, non-interventional, multicenter study aimed to define the distribution of different TMA forms in adult Turkish patients who were referred for therapeutic plasma exchange (TPE) for presumptive diagnosis of TMA. Patients with serum ADAMTS13 activity <5% were diagnosed as having acquired thrombotic thrombocytopenic purpura (aTTP). Patients presenting with ADAMTS13 activity 6-10 % / normal renal function and patients with ADAMTS13 activity >10 %, normal renal function and no secondary TMA were treated as unclassified TMA. The study included a total of 80 patients (women: 50; man: 30) with a median age of 48 (20-74). Detailed evaluation at 1 month after hospital admission revealed aTTP, secondary TMA, infection/complement-associated hemolytic uremic syndrome and unclassified TMA in 29 (36.2 %), 22 (27.5 %), 23 (28.8 %) and 6 (7.5 %) patients respectively. As subclassification of various TMAs will dictate specific therapy, proper diagnosis in a timely manner is of utmost clinical significance., (Copyright © 2022. Published by Elsevier Ltd.)

Thrombotic microangiopathy(TMA) is a pathological diagnosis characterized by abnormalities of small vessels leading to microvascular thrombosis of arterioles and capillaries. The current prospective, non-interventional, multicenter (n:18) study aimed to define distribution of different TMA forms in adult Turkish patients who were referred for therapeutic plasma exchange (TPE) for a presumptive diagnosis of TMA. Patients with serum ADAMTS13 activity <5% were diagnosed as acquired thrombotic thrombocytopenic purpura (aTTP). Patients presenting with ADAMTS13 activity 6-10 % / normal renal function and patients with ADAMTS13 activity >10 %, normal renal function and no secondary TMA were treated as unclassified TMA. The study included a total of 97 patients (female: 60; male: 30) with a median age of 48 (18-74). Detailed evaluation at 1 month after hospital admission revealed aTTP, secondary TMA, infection/complement-associated hemolytic uremic syndrome and unclassified TMA in 32 (33 %), 33 (34 %), 26 (27 %) and 6 (6%) patients respectively. As subclassification of various TMAs will dictate specific therapy, proper diagnosis in a timely manner is of utmost clinical significance., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Introduction: Venetoclax is a selective B-cell lymphoma 2 (BCL2) inhibitor, which is approved to treat elderly patients with newly diagnosed acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (MDS) in combination with either low-dose cytarabine (ARA-C) or hypomethylating agents. We aimed to collect and share data among the efficacy and safety of venetoclax both as a monotherapy or in combination with other drugs used to treat high-risk MDS or AML., Materials and Methods: A total of 60 patients with a median age of 67 (30-83) years from 14 different centers were included in the final analysis. Thirty (50%) of the patients were women; 6 (10%) of the 60 patients were diagnosed with high-risk MDS and the remaining were diagnosed with AML., Results: The best objective response rate (complete remission [CR], complete remission with incomplete hematological recovery (CRi), morphological leukemia-free state [MLFS], partial response [PR]) was 35% in the entire cohort. Best responses achieved during venetoclax per patient number were as follows: 7 CR, 1 CRi, 8 MLFS, 5 PR, and stable disease. Median overall survival achieved with venetoclax was 5 months in patients who relapsed and not achieved in patients who were initially treated with venetoclax. Nearly all patients (86.7%) had experienced a grade 2 or more hematologic toxicity. Some 36.7% of these patients had received granulocyte colony stimulating factor (GCSF) support. Infection, mainly pneumonia (26.7%), was the leading nonhematologic toxicity, and fatigue, diarrhea, and skin reactions were the others reported., Conclusion: Our real-life data support the use of venetoclax in patients with both newly diagnosed and relapsed high-risk MDS and AML., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare X-linked genetic disorder. On the contrary to its name, it is a multisystemic disease and various symptoms other than hemoglobinuria could be occurred. It could be life threatening especially because of thromboembolic events. In the last decade, a terminal complement inhibition with eculizumab approved with promising results for PNH patients. We conducted this study to evaluate the long term experience of eculizumab therapy from Turkey for the first time. Our cohort included 138 patients with PNH treated with eculizumab between January 2008 and December 2018 at 28 centers in Turkey. Laboratory and clinical findings at the time of diagnosis and after eculizumab therapy were recorded retrospectively. The median age was 39 (range 18-84) years and median granulocyte PNH clone size was 74% (range 3.06-99.84%) at the time of diagnosis. PNH with bone marrow failure syndrome was detected in 49 patients and the rest of 89 patients had classical PNH. Overall 45 patients (32.6%) had a history of any prior thrombotic event before eculizumab therapy and only 2 thrombotic events were reported during the study period. Most common symptoms are fatigue (75.3%), hemoglobinuria (18.1%), abdominal pain (15.2%) and dysphagia (7.9%). Although PNH is commonly related with coombs negativity, we detected coombs positivity in 2.17% of patients. Seven months after the therapy, increased hemoglobin level was seen and remarkably improvement of lactate dehydrogenase level during the treatment was occurred. In addition to previous studies, our real life data support that eculizumab is well tolerated with no serious adverse events and improves the PNH related findings., Competing Interests: None., (AJBR Copyright © 2021.)

Chronic anemia, transfusion-associated iron deposition, and chelating agents lead to renal impairment in β-thalassemia (β-thal) patients. The present study aimed to determine the most reliable and practical method in assessing and predicting renal injury in β-thal major (β-TM) patients. Therefore, we assessed the predictive values of urine β2-microglobulin (β2-MG) and neutrophil gelatinase-associated lipocalin (NGAL) levels, their ratios to urine creatinine, and serum endocan level. Sixty β-TM patients and 30 healthy controls were included. Renal functions of the patients and controls were evaluated by means of urine protein/creatinine ratio, urine β2-MG, urine NGAL, and serum endocan level. The β-TM and control groups were comparable in terms of the demographic characteristics. Of the β-TM patients, 26.7% had glomerular hyperfiltration and 41.7% had proteinuria. Compared with the control group, the β-TM group had significantly higher levels of urine protein/creatinine, urine β2-MG, urine β2-MG/creatinine, urine NGAL, urine NGAL/creatinine, and serum endocan. These parameters did not differ between the chelating agent subgroups in the patient group. Urine β2-MG/creatinine and NGAL/creatinine ratios were the parameters with high specificity in predicting proteinuria. There were significant correlations of urine β2-MG, urine NGAL, and serum endocan levels with serum ferritin concentration. Urine β2-MG/creatinine, NGAL/creatinine, and protein/creatinine ratios were correlated with each other in the patient group. Positive correlations of urine β2-MG, urine NGAL, and serum endocan levels with serum ferritin concentration indicated that iron deposition was associated with endothelial damage and renal injury.

Background: This study investigated the effect of matrix metalloproteinase (MMP)-9 and 10, and stress hyperglycaemia on the necessity of emergency renal replacement therapy (RRT) and mortality in nondiabetic geriatric patients with acute kidney injury (AKI)., Materials and Methods: The present observational and longitudinal study included 101 nondiabetic geriatric patients (age >65 years) with AKI. The serum levels of MMP-9 and MMP-10 were evaluated in these patients. Serum glucose level >140 mg/dL at the time of admission was accepted as stress hyperglycaemia., Results: The average age of patients was 81 ± 7.1 years. Stress hyperglycaemia was diagnosed in 34.6% of the cases; the majority of these cases were patients with high-serum urea, CRP, and chronic kidney disease. The average levels of MMP-9 and MMP-10 were found to be 199 ± 38 and 16.5 ± 7.5 ng/mL, respectively. Thirty-one cases (30.6%) mortality during hospitalization and 20 cases (20%) underwent emergency RRT. Multiregression analysis showed the serum urea (P < .001) and stress hyperglycaemia (P = .03) to be independently associated with mortality. Also, serum urea (P = .01), potassium level (P = .03), and MMP-10 levels (P = .03) were independently associated with the necessity of the emergency RRT. The MMP-9 levels exhibited no relation with the necessity of emergency RRT and mortality., Conclusion: Stress hyperglycaemia is a common condition among nondiabetic geriatric patients with AKI and is related to mortality. Serum MMP-10 levels serve as an important predictor of the necessity of emergency RRT in these patients., (© 2018 Stichting European Society for Clinical Investigation Journal Foundation.)

Thrombotic 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 ADAMTS13 activity/anti-ADAMTS13 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/CA-HUS), 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., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Iron overload is one of the major prognostic factor in thallassemia patients. We aimed to evaluate iron accumulation in the heart and liver by MRI in thalassemia major, thalassemia intermedia, and S-ß thalassemia patients and to examine its association with ferritin and hepcidin levels. Serum ferritin and hepcidin levels were recorded. Iron overload (IOL) in the heart and liver parenchyma was determined based on the standardized T2* and R2 values measured by MRI. The results were evaluated considering the tissue iron overload, serum ferritin and hepcidin levels. Comparing the 109 patients with the 30 healthy controls revealed the mean age: 24.4 ± 11 versus 31.2 ± 5 years, median levels of serum ferritin: 1693 versus 40 ng/mL, and hepcidin: 1.94 versus 0.355 ng/mL; p  < 0.001, respectively. Comparison of age, serum ferritin and hepcidin levels and MRI findings of the patients with or without IOL revealed that, ferritin and T2* were significantly different in the patients with IOL in cardiac tissue ( p  = 0.004 and p  < 0.001), and, age, ferritin and R2 were significantly different in the patients with IOL in liver tissue ( p  = 0.036, p  < 0.001 and p  < 0.001). The MRI-based T2* and R2 values were moderately and inversely correlated with serum ferritin (r = -0.37; p  < 0.001 and r = -0.46; p  < 0.001). No correlations were found between the MRI-based T2*, R2 values and serum hepcidin. A moderate and positive correlation existed between serum ferritin and hepcidin (r = 0.45; p  < 0.001). We considered that, enhanced intestinal iron absorption characterized by decreased serum hepcidin levels in the intervals between successive transfusions were resulted in iron accumulation in our patients.

Background: Abnormal heart rate recovery (HRR) is predictive of cardiac mortality. Autonomic abnormalities in beta-thalassemia major (TM) patients have been reported in previous studies. However, the importance of low HRR in exercise stress test in TM patients has not yet been ascertained. Therefore, this study will be the first of its kind in the literature., Methods: Exercise stress test was performed on 56 TM patients who were being treated at the Thalassemia Center of our hospital, along with 46 non-TM iron deficiency anemia (IDA) patients as a control group. Values for HHR were recorded at 1, 2, 3, 4 and 5 min, and HRR was calculated by the difference of heart rate at peak exercise and at a specific time interval following the onset of recovery., Results: All HRR values were found to be lower in TM patients compared to those in the IDA group. Exercise capacity [metabolic equivalents (METs)] was also found to be low in these patients (p < 0.001) as well. Total exercise time was significantly lower in the TM group compared to the IDA group (8.40 ± 1.7 min vs. 11.17 ± 1.51 min, p < 0.001). Exercise capacity (METs) was also lower in the TM group compared to the IDA group. Mean T2* value was 28.3 ± 13.7 ms in TM patients on magnetic resonance imaging (MRI). In addition, there are 18 TM patients with T2* value was < 20 ms., Conclusions: This study found that TM was independently associated with low HRR. Such a condition is an indicator of autonomic dysfunction in TM patients, since abnormal HRR is related to impaired autonomic response. In addition, impaired HRR may be a marker of early cardiac involvement in patients, whose T2* value is high on MRI. Modifying HRR with a cardiac rehabilitation program in TM patients with impaired HRR is a field open for further investigation.

PNH Education and Study Group (PESG) have been established in December 2013 as a non-profit, independent, medical organization www.pesg.org. Paroxysmal Nocturnal Hemoglobinuria (PNH) is a multi-systemic disease that should be treated with a multidisciplinary approach. Patients may apply to the clinics other than the hematology due to variability and diversity of clinical findings which lower the rate of diagnosis due to low awareness about PNH. PNH might be overlooked and diagnosis might be delayed. Regarding these, PESG was established with the collaboration of Immunology, Cardiology, Thorax Diseases (Pulmonology), Neurology, Gastroenterology, General Surgery and Urology specialists in addition to hematologists dealing with PNH. The PESG study group aims to increase the awareness about PNH, including training activities about PNH, strengthening the relations between clinics and planning of clinical studies as a goal. It is the first professional organization focusing on PNH, in Turkey.In this guideline, we want to facilitate the diagnosis attributes of physicians from all specializations that deal with PNH and its systemic complications. One can perceive this as a tailor made guideline of international guidelines but not a compilation.

Background: It is generally well-understood that iron-mediated cardiomyopathy is the major complication that can arise from beta thalassemia major (TM). Therefore, early diagnosis, risk stratification, and the effective treatment of beta TM patients are clinically important to optimize long-term positive outcomes., Methods: This study included 57 beta TM patients with a mean age of 25 ± 7 years. We determined the serum ferritin level, echocardiography, heart rate recovery (HRR), and cardiac magnetic resonance (CMR) T2* in all patients. CMR T2* findings were categorized as normal myocardium (T2* > 20 ms), and myocardial involvement (T2* ≤ 20 ms). HRR values at 1-5 min (HRR1-5) were recorded; Subsequently. HRR was calculated by subtracting the heart rate at each time point from the heart rate at peak exercise., Results: There was a significant negative correlation between the serum ferritin level and the cardiac T2* MRI findings (r = -0.34, p = 0.009). A similar result was found in the negative correlation between serum ferritin and all heart rate recovery values. There was a significant positive correlation between HRR1, HRR2, and HRR3 values, and CMR T2* (T2* heart rate recovery (HRR)1: r = 0.51, p < 0.001; T2* HRR2: r = 0.48, p < 0.001; T2* HRR3: r = 0.47, p < 0.001, respectively)., Conclusions: The serum ferritin level and echocardiography can be used to predict the presence of myocardial iron load in beta TM patients. Therefore, HRR can be used to screen beta TM patients, and the clinical use of HRR can be a predictive marker for autonomic dysfunction in beta TM patients., Key Words: Beta thalassemia major • Cardiac magnetic resonance T2* • Heart rate recovery • Iron overload • Serum ferritin level • Tissue Doppler imaging.

Extramedullary hematopoiesis (EMH) is a phenomenon in which hematopoietic cells are found in sites other than the bone marrow. It is usually observed in the liver and spleen but may occasionally be found within solid tumors. The current case report presents a 69-year-old female patient who presented with a renal cyst. Histopathological examination following surgical removal of the cyst revealed a lining of oncocytic- and chromophobe-type cells with capsular invasion and a mass forming EMH with evident bone trabeculae within the cyst wall. Circulating hematopoietic stem cells in the blood and their colonization within tissues is discussed in the present case report, emphasizing certain types of renal cell carcinoma.

Thrombotic thrombocytic purpura (TTP) is a life-threatening disorder. Without plasma exchange treatment (PET) the mortality rate is quite high. Double-filtration plasmapheresis is an alternative opportunity for TTP patients refractory to PET. Here we report our experience in a refractory TTP patient who was successfully treated by means of double-filtration plasmapheresis therapy., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

During the course of the disease a patient with systemic lupus erythematosus (SLE) may develop inflammation of one or more serous membranes, resulting in pleural, peritoneal, or pericardial effusion. Chylous ascites and chylothorax have rarely been described in patients with SLE. Therefore, in parallel with the analysis of blood samples, detailed analysis of the effusions should be carried out. Supportive measures are often needed to relieve the symptoms of chylothorax or chylous ascites together with the treatment of the primary disease. The available literature had reported just 4 cases of chylous ascites and/or chylothorax in association with SLE, and this patient presented here is one of the rare cases apart from the reported ones.

Background/aims: Endoscopic ultrasonography (EUS) has been shown to be superior to conventional CT in detecting and staging pancreatic cancer. We conducted a prospective trial to compare EUS and MDCT, in discriminating benign/malignant, in determining local and vascular invasion of a suspected pancreatic cancer and deciding its resectability., Methodology: The study was performed at the Gastroenterology Department of Izmir Ataturk Training and Research Hospital, from June 2009 to June 2010, all patients with suspected pancreatic and periampullary tumors referred to our department were enrolled. A total of 56 patients were evaluated at the beginning. Five patients having distal CBD tumor (n=2), gallbladder tumor (n=1) and papillary tumor (n=2) were excluded from the study. Analysis was done for the remaining 51 patients., Results: For the diagnosis of resectability/unresectability, EUS alone demonstrated a definite role in 4 (9%) of the 43 patients in confirming surgical and pathologic results and MDCT alone demonstrated a definite role in 6 (14%) of the 43 patients in confirming their results. An accurate diagnostic decision regarding resectability/unresectability was accomplished in 27 (63%) patients with EUS and MDCT., Conclusions: While MDCT is clinically quite effective, in terms of a correct resectability/unresectability decision, EUS should also be used.

Background: We aimed to examine the effect of transient hyperglycemia in non-diabetic patients with febrile neutropenia., Methods: A total of 86 patients with febrile neutropenia were evaluated between June 2006 and December 2009. After measuring random blood glucose level at admission, cases with stress hyperglycemia were included in the study. Stress hyperglycemia was defined as documented random blood glucose level of 140 mg/dl and above without known diabetes mellitus, impaired glucose tolerance and impaired fasting glucose. A Multinational Association for Supportive Care in Cancer (MASCC) scoring system was used for the prediction of low and high risk patients according to medical complications at the onset of the febrile episode., Results: There were more patients with stress hyperglycemia than the patients with normoglycemia in the high risk group (p = 0.001). The growth of gram negative bacteria and fungi was higher in patients with stress hyperglycemia than with normoglycemia (p = 0.001). The patients receiving antifungal therapy had a higher rate of stress hyperglycemia than the patients without receiving antifungal therapy (p = 0.009). The patients with stress hyperglycemia had higher mortality rates than the patients with normoglycemia (p = 0.007). According to the MASCC risk-index, stress hyperglycemia increased 3.35 fold in the high risk patients compared to the low risk patients (p = 0.046) and 4.14 fold in the patients treated with antibacterial and antifungal agents compared to the patients treated with only antibacterial agents (p = 0.038)., Conclusion: Patients with stress hyperglycemia had more adverse clinical outcomes than patients with normoglycemia. We think further studies are needed to evaluate the relationship between stress hyperglycemia and febrile neutropenia., (Copyright © 2011 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.)