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Aim: To determine the unmet needs and challenges in management, diagnosis, treatment, follow-up and patient-physician communication in acute leukemia (AL). Materials & methods: The study was based on a modified Delphi approach. A questionnaire including the major potential obstacles was circulated twice among 13 hematologists. Results: The obstacles in AL management were limited access to the novel treatments and genetic tests, limited bed capacity, insufficient level of knowledge among allied health personnel, limited availability of psycho-oncological support and low levels of awareness in the population about the importance of stem cell donation. Conclusion: The challenges in the management of AL are critical to guide the efforts to improve the quality of healthcare delivery and the evidence-based decision making at treatment of AL patients.

Introduction: In multiple myeloma cases, a variety of prognostic parameters have been identified, which contain the Durie-Salmon classification and the international staging system (ISS) that takes the serum ß2 microglobulin and albumin levels, platelet-to-lymphocyte ratio (PLR), neutrophil-to-lymphocyte ratio (NLR), and monocyte-to-lymphocyte ratio (MLR). This study investigates the effect of haemoglobin, albumin, lymphocyte and platelet (HALP) score which is a marker of inflammation status and nutrition, at the time of diagnosis for the patients with multiple myeloma on prognosis., Methods: A total of 200 multiple myeloma patients with HALP scores calculated from serum haemoglobin, albumin, lymphocyte count and platelet levels at the time of diagnosis were retrospectively examined. The effect of HALP score on overall survival (OS) and progression-free survival and its relationship between the previously evaluated prognostic parameters were investigated., Results: The optimal cut-off value with the ROC curves for the HALP score was 28.8. The patients were divided into two groups according to the optimal value of the HALP score (low-score group: HALP ≤28.8 [n: 134] and high-score group HALP >28.8 [n: 66]). In the group with the high HALP score, the OS was statistically longer than the low HALP score group (84 months and 53 months; p = 0.0001). In addition, when the effects of NLR, PLR, HALP score and ISS stage on OS were examined by multivariate analysis, all these markers were found to be statistically significant predictors., Conclusions: HALP score may be a valuable prognostic marker for patients with multiple myeloma., (© 2022 John Wiley & Sons Ltd.)

Tyrosine kinase inhibitors (TKIs) approved for chronic myeloid leukemia known to have similar efficacies but different safety profiles. Therefore, the choice of patient-specific treatments is driven by factors such as tolerability and adverse event profile of TKIs. This review article examines the most up-to-date data and provides practical recommendations for clinical approaches. Nilotinib and ponatinib should be avoided in patients with cardiovascular risk factors, dasatinib in patients with lung damage and bosutinib and nilotinib in patients with liver disease. Considering that certain comorbidities predispose some patients to developing severe adverse events when receiving TKIs, the first- and second-line treatment of chronic myeloid leukemia should be tailored to each patient's individual condition., Competing Interests: Financial & competing interests disclosure All authors received an honorarium from Pfizer in connection with the development of this study. G Saydam has acted in advisory board of Novartis, AbbVie, Amgen, Pfizer Pharmaceuticals and Bristol Myers Squibb and has received speaker honorarium from Novartis. R Ali has acted in advisory board of Novartis, Amgen, Pfizer Pharmaceuticals and Bristol Myers Squibb and has received speaker honorarium from Novartis, Amgen and Pfizer Pharmaceuticals. AM Demir has acted in advisory board of Novartis, AbbVie, Amgen, Pfizer Pharmaceuticals and Bristol Myers Squibb and has received speaker honorarium from Novartis. AE Eskazan has acted in advisory board of Novartis and Pfizer Pharmaceuticals, and has received speaker honorarium from Pfizer Pharmaceuticals, Bristol Myers Squibb and Novartis. B Guvenc has acted in advisory board of Novartis, AbbVie, Amgen, Pfizer Pharmaceuticals and Bristol Myers Squibb and has received speaker honorarium from Novartis. IC Haznedaroglu has acted in advisory board of Novartis, Pfizer Pharmaceuticals and Bristol Myers Squibb and has received speaker honorarium from Novartis. MA Ozcan has acted in advisory board of Novartis, AbbVie, Amgen, Pfizer Pharmaceuticals, and Bristol Myers Squibb and has received speaker honorarium from Novartis. O Salim has acted in advisory board of Novartis, AbbVie, Amgen, Pfizer Pharmaceuticals and Bristol Myers Squibb. M Sonmez has acted in advisory board of Novartis, AbbVie, Amgen, Pfizer Pharmaceuticals and Takeda and has received speaker honorarium from Novartis. M Turgut has acted in advisory board of Novartis, Pfizer Pharmaceuticals, and Bristol Myers Squibb and has received speaker honorarium from Novartis. ATT has acted in advisory board of Novartis, AbbVie, Pfizer Pharmaceuticals, and Takeda and has received speaker honorarium from these companies. A Unal has acted in advisory board of Novartis, AbbVie, Amgen, and Bristol Myers Squibb and has received speaker honorarium from Novartis. S Bozkurt, B Aver and B Ozdengulsun are employees in Pfizer Pharmaceuticals. O İlhan has acted in advisory board of Novartis, AbbVie, Amgen, Pfizer Pharmaceuticals, Bristol Myers Squibb, Janssen Pharmaceutical, Deva Holding, Nobel Ilac and Roche. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. Medical Writing support was provided by Ayşe Özlem Yılmaz and submission assistance was provided by İlknur Kehlibar at Remedium Consulting Group and was funded by Pfizer., (© 2022 Guray Saydam and all co-authors.)

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.)

Talacotuzumab, a humanized anti-CD123 monoclonal antibody, was evaluated in combination with decitabine in elderly patients with acute myeloid leukemia (AML) not eligible for intensive chemotherapy. A multicenter, phase 2/3 study was initiated to determine the recommended phase 2 dose (RP2D) of talacotuzumab (Part A) followed by an open-label, randomized comparison of talacotuzumab in combination with decitabine versus decitabine alone to assess achievement of complete response (CR) and overall survival (OS) in Part B. Ten patients were enrolled in Part A and 316 in Part B; the results presented here are based on a database lock on January 25, 2018. Part A confirmed the RP2D of talacotuzumab to be 9 mg/kg. In Part B, CR was achieved in 12/80 (15%) patients receiving combination therapy and in 9/82 (11%) patients receiving decitabine alone (odds ratio: 1.4; 95% confidence interval [CI]: 0.6-3.6; p = 0.44). Median (95% CI) OS was 5.36 (4.27-7.95) months for combination therapy versus 7.26 (6.47-8.64) months for decitabine alone (hazard ratio: 1.04; 95% CI: 0.79-1.37; p = 0.78). Combination therapy showed no improvement in efficacy versus decitabine alone, resulting in the Independent Data Monitoring Committee's recommendation of early termination of enrollment and discontinuation of talacotuzumab treatment.

Peripheral blood is the prefered source for hematopoietic stem cells for hematopoietic stem cell transplantation. The efficiency of peripheral blood stem cell (PBSC) collection can vary among devices. In this study we aimed to compare feasibility and effectivity of apheresis procedures of the different systems. Two apheresis systems [Com.Tec (Fresenius Healthcare) and Spectra Optia (Caridian BCT)] were used in our center for the collection of PBSCs for autologous and allogeneic transplantation. We retrospectively analysed 190 apheresis procedures performed in healthy donors and patients between June 2012 and November 2014 in Department of Hematology, Dokuz Eylul University. PBSCS were collected by Fresenius cell separator (64 procedure) or Spectra Optia cell separator (126 procedure). Mobilization treatments were G-CSF (26.8%), cyclophosphamide plus G-CSF (48.4%), prelixafor plus G-CSF (14.7%), ESHAP (10%) and others. Patient and donor characteristics (age, weight, volume processed, disease, mobilization regimes) were similar in Fresenius and Spectra Optia apheresis groups. Altough both collected PBSCs efficiently, the amount of CD34+ cell in product collected by Spectra Optia device was significantly higher ( p  < 0.05) and product volume was lower than Fresenius Com.Tec significantly ( p  < 0.05). "CD34+ collection efficiency" with Spectra Optia was significantly higher than Fresenius Com.Tec (CE2: 87%, 70%, p  = 0.033) regarding all procedures. High collection efficiency and low product volume may be a significant characteristic of Spectra Optia device (mean 187 mL, product CD34+ cell: 1576 µL)., Competing Interests: Compliance with Ethical StandardsThe author did not receive financial compensation for authoring the manuscript.

Background: Recent reports showed neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and monocyte-to-lymphocyte ratio (MLR), as a predictor of progression-free survival (PFS) and overall survival (OS) in various malignancies., Materials and Methods: We retrospectively examined the PLR, NLR, and MLR in a cohort of 186 newly diagnosed multiple myeloma (MM) patients. This study investigated the prognostic relevance of NLR, PLR, and MLR in MM patients. NLR, PLR, and MLR were calculated from whole blood counts before therapy. The Kaplan-Meier curves and multivariate Cox models were used for the evaluation of survival., Results: Applying cutoff of 1.9 (NLR), 120.00 (PLR), and 0.27 (MLR), decreased PLR showed a negative impact on the outcome. Decreased PLR is an independent predictor for PFS and OS. There were no significant differences in median survival between the high and low NLR ( P = 0.80) and MLR ( P = 0.87) groups., Conclusions: In this study, thrombocytopenia and low PLR are associated with poor survival in MM patients does this P value apply to thrombocytopenia or low PLR and may serve as the cost-effective prognostic biomarker., Competing Interests: There are no conflicts of interest.

Background: Recent reports have showed that neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and monocyte-to-lymphocyte ratio (MLR) are predictors of progression-free survival (PFS) and overall survival (OS) in many types of cancer. This study evaluates the predictive value of NLR, MLR, and PLR for survival in MM patients treated with to ASCT., Methods: A set of data consisting of 150 patients who underwent autologous stem cell transplantation (ASCT) for MM was collected retrospectively. The prognostic value of NLR, MLR, and PLR was investigated with Kaplan-Meier method., Results: The prognostic value of NLR, MLR, and PLR was analyzed by a receiver operating characteristic (ROC) curve established to determine the cutoff. These cutoff values of NLR, PLR, and MLR were found 1.46, 86, and 0.27, respectively, on the 100th day of post-transplantation period. The overall survival (OS) and the post-transplantation OS of the patients with high NLR, MLR, and PLR levels on the 100th day of post-transplantation were shorter than the other group (P = 0.05, P = 0.018 [NLR], P = 0.05, P = 0.002 [MLR], P = 0.000, P = 0.001 [PLR]). The post-transplantation progression-free survival (PFS) of the patients with high NLR, MLR, and PLR levels on the 100th day of post-transplantation was shorter as well (P = 0.036, P = 0.001, P = 0.001, respectively)., Conclusion: As increased NLR, MLR, and PLR predicted poor clinical outcome in MM patients with autologous transplantation in this study, they may serve as cost-effective and rapidly available prognostic biomarkers for these patients., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

We describe a 36-year-old woman with a relapsing Hodgkin's lymphoma who developed a severe bilateral sudden visual loss almost three weeks after the initiation of brentuximab therapy. Ancillary fundus tests yielded bilateral severe retinal arteriolar occlusion 360° and serous macular retinal detachment. No visual improvement could be achieved despite the pulse corticosteroid therapy and a single bilateral intravitreal aflibercept administration cessation of the brentuximab therapy. Unfortunately, she succumbed to respiratory failure almost six weeks after the diagnosis of Purtscher-like retinopathy., Competing Interests: The authors declare that they have no competing interests.

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.

Multiple myeloma (MM) is a hematologic cancer characterized by malignant proliferation of plasma cells and their precursors. Immunosuppressive CD4+CD25+Foxp3+ regulatory T (Treg) cells are increased in the peripheral blood of patients with MM. On the basis of this finding, we sought to evaluate the ex vivo effect of CD4+CD25+Foxp3+ Treg cells on the anti-tumor effect of the proteosome inhibitor bortezomib on MM cells. We collected peripheral blood and bone marrow aspiration samples from 20 patients with newly diagnosed MM and isolated CD4+CD25+Foxp3+ Treg cells from peripheral blood mononuclear cells. The bone marrow mononuclear cells were cultivated in RPMI at 37°C and 5% CO2 for 72 hours. The LD50 doses of bortezomib, isolated Treg cells, and their combination were added. After 24 hours, the viability of CD138+ myeloma cells was evaluated by WST-1. We compared the anti-tumor effect of bortezomib alone and in combination with Treg expansion and statistically analyzed the measured differences with respect to the clinical parameters of the patients. Treg cells had varied effects on bortezomib, increasing, decreasing, or not changing its anti-tumor effect. The increased in vitro anti-tumor effect of bortezomib after Treg cell expansion was correlated in patients who did not develop bortezomib resistance in vivo (p = 0.022). These patients with in vivo non-bortezomib-resistant MM also responded to Treg expansion with decreased cell viability (p = 0.024). Our data indicate that the ex vivo expansion of Treg cells increased the cytotoxic effect of bortezomib in clinically sensitive cases., (Copyright © 2016 ISEH - International Society for Experimental Hematology. Published by Elsevier Inc. All rights reserved.)

Introduction: Essential thrombocythemia (ET) is the most common of the myeloproliferative neoplasms. For better predicting the occurrence of thrombotic events, an International Prognostic Score of Thrombosis for ET (IPSET-Thrombosis) was recently developed. We aimed to investigate the validity of IPSET-Thrombosis in a Turkish patient cohort and to compare the efficacy of IPSET-Thrombosis and conventional risk scoring systems in predicting thrombosis-free survival., Patients and Methods: We retrospectively evaluated the clinical characteristics and risk factors for thrombosis in 112 Turkish patients. Median thrombosis-free survival and Harrell C concordance indexes were calculated for both conventional and IPSET-Thrombosis., Results: Median age of 112 patients included in the study was 61 (range, 27-90) years at the time of diagnosis. When patients were stratified according to the conventional risk stratification system, 43.8% of patients were in the low-risk group and 56.2% in the high-risk group. A total of 22.4% of low-risk and 42.9% of high-risk patients had at least one thromboembolic event. When patients were stratified according to the IPSET-Thrombosis, 33% were in the low-risk group, 26.8% in the intermediate-risk group, and 40.2% in the high-risk group. Considering IPSET-Thrombosis risk groups, 5.4% of low-risk, 26.7% of intermediate-risk, and 66.2% of high-risk patients had at least one thromboembolic event. Regarding IPSET-Thrombosis risk groups, 10-year thrombosis-free survival was 86.8% for low-risk, 39.4% for intermediate-risk, and 32.9% for high-risk groups (P < .001). Harrell C concordance indexes of conventional and IPSET-Thrombosis were 0.60 and 0.77, respectively., Conclusion: By validating the reproducibility of IPSET-Thrombosis in Turkish ET patients, we found that IPSET-Thrombosis identifies thrombosis-free survival better than the conventional risk stratification system., (Copyright © 2015 Elsevier Inc. All rights reserved.)