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Background: Therapeutic plasma exchange (TPE) is a technique in which plasma is separated from the rest of the blood in an extracorporeal system and exchanged with appropriate fluids. Two main methods are used in the TPE process: membrane filtration (mTPE) and centrifuge-based (cTPE) TPE. We aimed to compare the efficacy of these methods and their effects on hemostatic parameters., Methods: A total of 88 TPE procedures performed on 51 patients were evaluated retrospectively. Hemostatic parameters, such as pre- and postoperative complete blood count, fibrinogen, and D-dimer levels were evaluated, as well as data recorded during the TPE application, such as preparation time, operation time, and plasma removal efficiency (PRE). The Fresenius multiFiltrate, software version 5.3 device was used for the mTPE procedure and the Spectra Optia device was used for cTPE., Results: While both modalities removed similar amounts of plasma, the total time to perform the cTPE treatment was significantly lower than the mTPE (107 [66-191] min vs 116.5 [80-181] min, respectively) (P = .026). At the PRE rate, the mTPE procedure was as effective as cTPE (86.8% ± 16.3 vs 85.15% ± 17.63 respectively, P = .64). The difference was not statistically significant, and this is the highest rate of PRE reported about the mTPE procedure in the literature., Conclusions: To our knowledge, this is the first study directly comparing Fresenius and Spectra regarding mTPE and cTPE. While there were no statistically significant differences regarding PRE, treatment time of the mTPE was significantly longer than the cTPE treatment time., (© 2022 Wiley Periodicals LLC.)

Secondary hypogammaglobulinemia (SHG) is characterized by a decrease in total serum immunoglobulin (Ig) levels and can lead to immunodeficiency associated with recurrent and severe infections and is a common complication of chronic lymphocytic leukaemia (CLL). SHG also increases with the treatment of CLL. Ibrutinib is one of these treatments and acts by inhibiting bruton tyrosine kinase. Twenty-seven patients with relapsed/refractory (R/R) CLL who received ibrutinib monotherapy were included. IgG levels, stage, bulky disease, previous treatments, genetics and laboratory features, overall survival (OS) and progression free survival (PFS) were compared with and without SHG. Nine patients (33.3%) had SHG and 18 patients (66.6%) didn't have SHG. The mean IgG levels after ibrutinib treatment first, third, 6th and 12th months were 684, 531.3, 452 and 360 mg/dL respectively in SHG arm ( p  < 0.001) and 1156, 1058.2, 1012.8 and 886.9 mg/dL respectively in without SHG arm ( p  < 0.001). All patients with SHG had ibrutinib related other adverse effects(AEs) but 2 (11.1%) patients without SHG had AEs ( p  < 0.001). In SHG arm 7 (77.7%) had complete and partial remission but in other arm only 6 (33.3%) had ( p : 0.029). There was no significant difference in OS and PFS ( p values 0.95 and 0.64, respectively). IgG levels at the beginning of ibrutinib treatment is the best predicted value for SHG development in our study ( p  = 0.001). As a result, we reported a significant decrease in IgG values after ibrutinib monotherapy in R/R CLL patients. This decrease occurs every month after ibrutinib use, but after a maximum of 1 year., Supplementary Information: The online version contains supplementary material available at 10.1007/s12288-021-01466-1., Competing Interests: Conflict of interestThe authors report no conflict of interest., (© Indian Society of Hematology and Blood Transfusion 2021.)

Objective: Patients with solid malignancies are more vulnerable to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection than the healthy population. The outcome of SARS-CoV-2 infection in highly immunosuppressed populations, such as in patients with hematological malignancies, is a point of interest. We aimed to analyze the symptoms, complications, intensive care unit admissions, and mortality rates of patients with hematological malignancies infected with SARS-CoV-2 in Turkey., Materials and Methods: In this multicenter study, we included 340 adult and pediatric patients diagnosed with SARS-CoV-2 from March to November 2020. Diagnosis and status of primary disease, treatment schedules for hematological malignancies, time from last treatment, life expectancy related to the hematological disease, and comorbidities were recorded, together with data regarding symptoms, treatment, and outcome of SARS-CoV-2 infection., Results: Forty four patients were asymptomatic at diagnosis of SARS-CoV- 2 infection. Among symptomatic patients, fever, cough, and dyspnea were observed in 62.6%, 48.8%, and 41.8%, respectively. Sixty-nine (20%) patients had mild SARS-CoV-2 disease, whereas moderate, severe, and critical disease was reported in 101 (29%), 71 (20%), and 55 (16%) patients, respectively. Of the entire cohort, 251 (73.8%) patients were hospitalized for SARS-CoV-2. Mortality related to SARS-CoV-2 infection was 26.5% in the entire cohort; this comprised 4.4% of those patients with mild disease, 12.4% of those with moderate disease, and 83% of those with severe or critical disease. Active hematological disease, lower life expectancy related to primary hematological disease, neutropenia at diagnosis of SARS-CoV-2, ICU admission, and first-line therapy used for coronavirus disease-2019 treatment were found to be related to higher mortality rates. Treatments with hydroxychloroquine alone or in combination with azithromycin were associated with a higher rate of mortality in comparison to favipiravir use., Conclusion: Patients with hematological malignancy infected with SARS-CoV-2 have an increased risk of severe disease and mortality.

Imatinib mesylate, a tyrosine kinase inhibitor, is the first choice in chronic myeloid leukemia treatment. However, resistance to imatinib may develop with time and in some cases, patients may not respond at all to imatinib. Progressive resistance to imatinib therapy is often due to mutations in the BCR/ABL region. Within the scope of our study 124 patients were evaluated via pyrosequencing between 2015 and 2020. In this regard, 32 patients who have a partial response and have no response to imatinib therapy were included in the study. In addition, next-generation sequencing (NGS) analysis was performed on 15 patients who were resistant to imatinib treatment according to the molecular follow-up reports. With pyrosequencing, 5 cases out of a total of 124 were found to be positive. This means that approximately 4.03% of the proportion is positive. But when we examined only 32 patients who have a partial response and have no response to imatinib therapy this rate is rising 15.6%. NGS analysis was performed with 15 patients who have no mutation with pyrosequencing of 32 patients and VUS (Variant of Uncertain Significance) mutation was detected in one. In this study, our aim was to determine the mutations of the BCR/ABL and to evaluate the mutations by NGS and pyrosequencing. Our study is important in terms of comparing the pyrosequencing with NGS mutation rates, drawing attention to the clinical importance of log reduction., (© 2021. Springer Science+Business Media, LLC, part of Springer Nature.)

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.

Autologous stem cell transplantation (ASCT), supported by high-dose chemotherapy, is the prevalent option for multiple myeloma (MM) treatment in candidates suitable for transplantation. Although granulocyte colony-stimulating factor (G-CSF) supported cyclophosphamide (CY) is used as the pre-ASCT mobilization regimen, there is no consensus on the optimal dosage of CY. Thus, in this study, we examined the results of 47 MM patients, who underwent ASCT after mobilization with intermediate (ID) or low-dose (LD) CY treatment supported with G-CSF. As the mobilization regimen, we used ID (2.4 g/m 2 ) of CY in 22 patients, and LD (1 g/m 2 ) of CY in 25 patients. Adequate doses of CD34+ cells were collected in both groups. At the same time, febrile neutropenia was observed to be less common in patients in the LD-CY group. Additionaly 96% of patients in LD-CY group did not need to be hospitalized during the mobilization. In conclusion, we think that mobilization with LD-CY and G-CSF is advantageous since it results in a sufficient amount of stem cells in addition to being advantageous in terms of patient safety and cost., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background/aim: PD-1 (programmed death-1) is an immune checkpoint receptor that modulates T-cell activity in peripheral tissues via interaction with its ligands, PD-L1 (programmed death-ligand 1) and PD-L2 (programmed death-ligand 2). Tumor cells upregulate PD-L1 or PD-L2 to inhibit this T lymphocyte attack. Our goal was to determine the PD-1 and PD-L2 expression rates of various hematologic malignancies, and evaluate whether PD-1 and PD-L2 expressions have an impact on prognosis., Materials and Methods: For this purpose, pretreatment bone marrow biopsy specimens of 83 patients [42 multiple myeloma (MM), 21 acute leukemia, and 20 chronic lymphocytic leukemia (CLL)] were stained with monoclonal antibody immunostains of PD-1 and PD-L2., Results: As a result, the overall expression rate of PD-1 was 26.2%, 4.8%, and 60% in patients with MM, acute leukemia, and CLL, respectively, whereas the PD-L2 expression rate was 61.9%, 14.3%, and 10% in patients with MM, acute leukemia, and CLL, respectively., Conclusion: Finally, we concluded that the role of the PD-1 pathway can be demonstrated by immunohistochemistry (IHC). Since we evaluated whether there is a correlation between the (IHC) results and survival of patients with MM, acute leukemia, and CLL, we could not demonstrate meaningful evidence that these markers have an impact on prognosis.

Aspergillus lateral-flow device (LFD) was recently introduced as a practical tool for the diagnosis of invasive aspergillosis (IA). We investigated the performance of Aspergillus -LFD as a point-of-care test for the diagnosis of IA. Serum samples were collected twice weekly from patients who received intensive chemotherapy for acute leukemia, or recepients of allogeneic stem cell transplantation. Aspergillus galactomannan (GM) antigen, 1,3-beta-d-glucan and Aspergillus -LFD tests were carried out according to manufacturers' recommendations. GM testing was repeated with a modified procedure which was proven to increase the sensitivity. Aspergillus -LFD was performed without applying any pretreatment procedure to allow the kit to fit as a point-of-care test. Fungal infections were categorized according to European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) criteria. A total of 75 neutropenia episodes in 64 patients were prospectively followed between February 2012 and January 2013. Probable IA was diagnosed in 11 patients, probable pulmonary fungal disease was diagnosed in one patient, and rhinocerebral aspergillosis was diagnosed in one patient. Fungemia was detected in two patients. Aspergillus -LFD was positive in serum of a patient with probable IA and in the bronchoalveolar lavage fluid of an other patient with probable IA. Aspergillus -LFD was false positive in serum of two patients. Although there was no radiological finding of IA or documented fungemia, fever resolved after empirical caspofungin therapy in one of these patients. The sensitivity of Aspergillus -LFD as a point-of-care test without any pretreatment of serum sample is low., Competing Interests: Compliance with Ethical Standards Conflicts of interest G.M. has received honarium for speaking at symposia and lecture organized by Gilead, financial compensation from Pfizer for the time and expenses for a meeting organised to discuss the content of a review paper, member of advisory board of Pfizer and Astellas, and received travel Grants form MSD, Pfizer, and Gilead to participate conferences. L.K. has recieved honarium for speaking at a lecture organized by Gilead. The other authors declared to have any completing interest.

Blood component donations by apheresis have become more common in modern blood transfusion practices. We compared three apheresis instruments (Fenwal Amicus, Fresenius COM.TEC, and Trima Accel) with regard to platelet (PLT) yield, collection efficiency (CE), and collection rate (CR). The single-needle or double-needle plateletpheresis procedures of the three instruments were compared in a retrospective, randomized study in 270 donors. The blood volume processed was higher in the COM.TEC compared with the Amicus and Trima. Also there was a significantly higher median volume of ACD used in collections on the COM.TEC compared with the Amicus and Trima. The PLT yield was significantly lower with the COM.TEC compared with the Amicus and Trima. Additionally, the CE was significantly lower with the COM.TEC compared with the Amicus and Trima. There was no significant difference in median separation time and CR between the three groups. When procedures were compared regarding CE by using Amicus device, it was significantly higher in single-needle than double-needle plateletpheresis. When double-needle Amicus system was compared with double-needle COM.TEC system, CE and PLT yield were significantly higher with Amicus system. When single-needle Amicus system was compared with single-needle Trima system, CE and PLT yield were significantly higher with Trima system. All instruments collected PLTs efficiently. However, the CE was lower with the COM.TEC compared with the Amicus and Trima. Also, we found Amicus single-needle system collected PLTs more efficiently compared with the double-needle system. CE and PLT yields were significantly higher with the single-needle Trima instrument compared with the single-needle Amicus device., (© 2014 Wiley Periodicals, Inc.)

Introduction: Iron overload (IO) has been shown to be an important cause of mortality and morbidity in patients who underwent allogeneic hematopoietic stem cell transplantation (alloHSCT). This study aimed to evaluate the possible effect of oral iron-chelation treatment (deferasirox) on survival in alloHSCT recipients in the posttransplant period., Materials and Methods: A total of 80 alloHSCT recipients with IO were analyzed, retrospectively. Pretransplant and posttransplant data were obtained from the patients' files. Patients were divided into two groups. Group 1; patients who did not receive any chelator treatment due to side effects or compliance problems. These patients were treated by phlebotomy. Group 2 consisted of patients who received deferasirox treatment., Results: The median treatment duration with deferasirox was 122 days (min-max:91-225). The iron chelating treatment significantly reduced serum ferritin levels administered at a dosage of 20-30 mg/kg/day (p<0.001). The median OS in Group 1 was found 16.0 (min-max:1.0-63.0) months and 25.0 (min-max:3.0-72.0) months in Group 2. In univariate and multivariate analysis, patients in Group 1 showed poorer OS compared to those in Group 2 with an increase in risk of death (HR:3.22, min-max:1.67-6.23, p=0.001 and HR:3.51,, min-max:1.75-6.99, p<0.001; respectively). The median DFS in Group 1 was found 11.0 (min-max:3.0-24.0) months and 22.0 (min-max:8.0-43.0) months in Group 2. The difference was found statistically significant (p=0.023). The other factors that we found significant difference in multivariate analysis between groups were; presence of acute GVHD (patients with aGVHD had increased risk of death compared to patients without aGVHD (HR:2.49, min-max: 1.32-4.69, p=0.005), chronic GVHD (HR:2.57, min-max:1.23-5.41, p=0.013), median interval to tx (HR: 2.23, min-max:1.17-4.26, p=0.015) and HLA match (HR:3.01, min-max:1.35-6.73, p=0.007) CONCLUSION: Oral deferasirox (Exjade) treatment may improve survival in patients with iron overload who underwent alloHSCT., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Therapeutic plasma exchange (TPE), is a procedure, changing pathologic substances in the plasma of patients with replacement fluid. TPE has an increasing list of indications in recent years such as neurological, connective tissue, hematological, nephrological, endocrinological and metabolic disorders. We report our multicenter data about therapeutic plasma exchange in patients with neurological diseases. Six University Hospitals' aphaeresis units medical records about neurologic diseases were reviewed retrospectively. Hundred and fifteen patients and 771 TPE sessions from six aphaeresis units' were included to this study. Of the 115 patients, 53 (46%) were men and 62 (54%) were women. The median age was 50 (range: 5-85) years. Of these patients 58.3% were Guillain-Barre syndrome (GBS), 17.4% were acute disseminated encephalomyelitis (ADEM), 10.4% were chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 7% were multiple sclerosis, 6.1% were myasthenia gravis (MG) and 0.9% were Wilson disease (WD). The median number of TPE sessions per patient was 5 (range 1-72). Human albumin was used as a replacement fluid in 66% and fresh frozen plasma was used in 34% of cases. TPE was done through central venous catheters in 66%, and peripheral venous access in 34% of patients. Some complications were seen in patients (18.3%) during TPE sessions. These complications were, complications related to catheter placement procedure (8.7%), hypotension (3.5%), hypocalcaemia (3.5%) and allergic reactions (1.7%). The complication ratios were 2.7% in total 771 TPE procedures. TPE procedure was terminated in 6% of sessions depending on these complications. Overall responses to TPE were noted in 89.5% of patients. In conclusion; Therapeutic plasma exchange is an effective treatment option in several neurologic diseases., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Unlabelled: Thrombotic thrombocytopenic purpura (TTP) is a particular form of thrombotic microangiopathy typically characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological abnormalities, and renal dysfunction. TTP requires a rapid diagnosis and an adapted management in emergency. Daily sessions of therapeutic plasma exchange (TPE) remain the basis of management of TTP. Also, TTP is a rare disease that is fatal if it is not treated. TPE has resulted in excellent remission and survival rates in TTP patients., Aim: We aimed to present our experience in 163 patients with TTP treated with TPE during the past 5years from 10 centers of Turkey., Patients and Methods: One hundered and sixty-three patients with TTP treated with TPE during the past 5years from 10 centers of Turkey were retrospectively evaluated. TPE was carried out 1-1.5times plasma volume. Fresh frozen plasma (FFP) was used as the replacement fluid. TPE was performed daily until normalization of serum lactate dehydrogenase (LDH) and recovery of the platelet count to >150×10(9)/dL. TPE was then slowly tapered. Clinical data, the number of TPE, other given therapy modalities, treatment outcomes, and TPE complications were recorded., Results: Fifty-eight percent (95/163) of the patients were females. The median age of the patients was 42years (range; 16-82). The median age of male patients was significantly higher than female (53 vs. 34years; p<0.001). All patients had thrombocytopenia and microangiopathic hemolytic anemia. At the same time, 82.8% (135/163) of patients had neurological abnormalities, 78.5% (128/163) of patients had renal dysfunction, and 89% (145/163) of patients had fever. Also, 10.4% (17/163) of patients had three of the five criteria, 10.4% (17/163) of patients had four of the five criteria, and 6.1% (10/163) of patients had all of the five criteria. Primary TTP comprised of 85.9% (140/163) of the patients and secondary TTP comprised of 14.1% (23/163) of the patients. Malignancy was the most common cause in secondary TTP. The median number of TPE was 13 (range; 1-80). The number of TPE was significantly higher in complete response (CR) patients (median 15.0 vs. 3.5; p<0.001). CR was achieved in 85.3% (139/163) of the patients. Similar results were achieved with TPE in both primary and secondary TTP (85% vs. 87%, respectively; p=0.806). There was no advantage of TPE+prednisolone compared to TPE alone in terms of CR rates (82.1% vs. 76.7%; p=0.746). CR was not achieved in 14.7% (24/163) of the patients and these patients died of TTP related causes. There were no statistical differences in terms of mortality rate between patients with secondary and primary TTP [15% (21/140) vs. 13% (3/23); p=0.806]. But, we obtained significant statistical differences in terms of mortality rate between patients on TPE alone and TPE+prednisolone [14% (12/86) vs. 3% (2/67), p<0.001]., Conclusions: TPE is an effective treatment for TTP and is associated with high CR rate in both primary and secondary TTP. Thrombocytopenia together with microangiopathic hemolytic anemia is mandatory for the diagnosis of TTP and if these two criteria met in a patient, TPE should be performed immediately., (Copyright © 2013. Published by Elsevier Ltd.)

Hyperbilirubinemia is a common clinical manifestation, and may be life threatening. Many diseases result in hyperbilirubinemias, some are refractory, and cannot be cured by medication or surgery. Plasma exchange (PE) for hyperbilirubinemia is not a pathogenesis oriented therapy but strives for the opportunity to cure. In the present study, we aimed to present the outcomes of treatment of hyperbilirubinemia with PE in patients with various disorders. Eleven patients who underwent PE due to hyperbilirubinemia between 2006 and 2012 in Apheresis Unit of Erciyes University, were retrospectively reviewed. After PE, we observed a marked decline in total and direct bilirubin levels. The decline in the biochemical values were statically significant (p<0.003). Both total and direct bilirubin levels remained above the normal limits in one of 11 patients. PE should be considered as an effective and safe option in cases with hyperbilirubinemia, and this procedure can improve survival in patients with sufficient residual capacity of liver regeneration., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Hyperthyroidism characterized by elevated serum levels of circulating thyroid hormones. The aim of hyperthyroidism treatment is to achieve a euthyroid state as soon as possible and to maintain euthyroid status. However, drug withdrawal and utilization of alternative therapies are needed in cases in which leucopenia or impairment in liver functions is observed during medical therapy. In the present study, we aimed to present our cases which underwent therapeutic plasma exchange (TPE) due to severe hyperthyroidism. The results of 22 patients who underwent therapeutic plasma exchange due to hyperthyroidism in Apheresis Units of Erciyes University and Gaziantep University, between 2006 and 2012, were retrospectively reviewed. These cases had severe thyrotoxic values despite anti-thyroid drug use. After TPE, we observed a significant decrease in free thyroxin (FT4) (p<0.001) and free triiodotyhronin (FT3) (p<0.004) levels. There was statistically significant increase in the mean values of TSH levels after TPE (p<0.001). Clinical improvement was achieved in hyperthyroidism by TPE in 20 cases (91%). Both FT3 and FT4 levels remained above the normal limits in two of 22 patients. TPE should be considered as an effective and safe therapeutic option to achieve euthyroid state before surgery or radioactive iodine treatment. TPE is a useful option in cases with severe hyperthyroidism unresponsive to anti-thyroid agents and in those with clinical manifestations of cardiac failure and in patients with severe adverse events during anti-thyroid therapy., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Background/aims: Acute variceal bleeding is the most severe consequence of portal hypertension. Mortality due to bleeding among cirrhotic patients is high; between 30 and 50% die within six weeks of the first bleeding episode. This dismal outcome has led to attempts both to stop acute bleeding and to prevent rebleeding. The aim of this study was to investigate the efficacy of subcutaneous octreotide treatment, administered after emergency sclerotherapy, in preventing rebleeding of esophageal varices, Methods: After a bolus injection of 50 microgram octreotide, 34 patients, forming the standard therapy (ST) group, received octreotide infusion at a rate of 50 microgram/h until endoscopic sclerotherapy performed within 36 hours. The same procedure was applied to another 27 patients in the maintenance therapy (MT) group in which octreotide was given at 100 microgram/8h via subcutaneous (sc) route after sclerotherapy for five days. In both groups, sclerotherapy was repeated on the 5th-7th day. Patients were followed for three weeks for rebleeding., Results: Nine patients rebled in the ST group but only one patient bled in the MT group (3.7% vs. 26.5% vs. 3.7%; p<0.05). Transfusion requirement and duration of hospitalization period were similar in both groups., Conclusions: This study suggests that maintenance subcutaneous octreotide therapy is effective in controlling rebleeding episodes.