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Uric acid (UA) acts as a richly available water-soluble antioxidant, contributing to approximately two-thirds of the overall free-radical-scavenging activity in human serum. It is discharged from damaged cells during the preparation for allogeneic hematopoietic stem cell transplantation (allo-HSCT). This study assessed how pretransplantation uric acid levels influence survival and mortality in allo-HSCT patients. In a retrospective analysis of 267 patients with hematologic diseases who underwent allo-HSCT, uric acid levels were documented on the day of allo-HSCT (day 0). Patients were divided into two cohorts based on their pretransplant uric acid levels: those at or below 4.4 mg/dL and those above 4.4 mg/dL. Among them, 153 (57.3%) were male, and 114 (42.7%) were female, with a median age of 31 years (ranging from 13 to 66). Those with serum uric acid levels of 4.4 mg/dL or lower exhibited notably poorer overall survival (OS) and disease-free survival (DFS) compared to those surpassing 4.4 mg/dL (p=0.032 and p=0.045, respectively). Our findings indicate an interrelation between low pretransplant serum uric acid levels and reduced survival rates in allo-HSCT patients. Further exploration into potential mechanisms, such as compromised antioxidative capacity in hypouricemia, may establish uric acid as a promising prognostic marker in allo-HSCT. [ABSTRACT FROM AUTHOR]

Graft versus host disease (GvHD) remains a significant risk for mortality and morbidity following allogeneic hematopoietic stem cell transplantation (HSCT). A growing literature supports successful applications of mesenchymal stromal cells (MSCs) for the treatment of steroid-refractory acute GvHD (aGvHD). However, there is limited knowledge about the effects of MSC treatment on late-acute GvHD (late aGvHD). In this article, we present our multicenter study on the safety and efficacy of MSC therapy for patients with steroid-refractory late aGvHD in comparison to those with aGvHD. The outcome measures include non-relapse mortality (NRM) and survival probability over a 2-year follow-up. The study includes a total of 76 patients with grades III-IV aGvHD (n = 46) or late aGvHD (n = 30), who had been treated with at least two lines of steroid-containing immunosuppressive therapy. Patients received weekly adipose or umbilical cord-derived MSC infusions at a dose of median 1.55 (ranging from 0.84 to 2.56) × 106/kg in the aGvHD group, and 1.64 (ranging from 0.85 to 2.58) × 106/kg in the late aGvHD group. This was an add-on treatment to ongoing conventional pharmaceutical management. In the aGvHD group, 23 patients received one or two infusions, 20 patients had 3–4, and three had ≥ 5. Likewise, in the late aGvHD group, 20 patients received one or two infusions, nine patients had 3–4, and one had ≥ 5. MSC was safe without acute or late adverse effects in 76 patients receiving over 190 infusions. In aGvHD group, 10.9% of the patients had a complete response (CR), 23.9% had a partial response (PR), and 65.2% had no response (NR). On the other hand, in the late aGvHD group, 23.3% of the patients had CR, 36.7% had PR, and the remaining 40% had NR. These findings were statistically significant (p = 0.031). Also, at the 2-year follow-up, the cumulative incidence of NRM was significantly lower in patients with late aGvHD than in patients with aGvHD at 40% (95% CI, 25–62%) versus 71% (95% CI, 59–86%), respectively (p = 0.032). In addition, the probability of survival at 2 years was significantly higher in patients with late aGvHD than in the aGvHD group at 59% (95% CI, 37–74%) versus 28% (95% CI, 13–40%), respectively (p = 0.002). To our knowledge, our study is the first to compare the safety and efficacy of MSC infusion(s) for the treatment of steroid-resistant late aGVHD and aGVHD. There were no infusion-related adverse effects in either group. The response rate to MSC therapy was significantly higher in the late aGvHD group than in the aGvHD group. In addition, at the 2-year follow-up, the survival and NRM rates were more favorable in patients with late aGVHD than in those with aGVHD. Thus, the results are encouraging and warrant further studies to optimize MSC-based treatment for late aGVHD. [ABSTRACT FROM AUTHOR]

Inflammatory processes play an important role in the pathophysiology of cGVHD. Serum fibrinogen is a proinflammatory protein with a wide range of functions in inflammation. But its role in GVHD is unclear. The aim of this study was to evaluate the predictive impact of pre-transplantation fibrinogen levels on cGVHD in allo-HSCT recipients. We retrospectively analyzed 249 patients with hematologic diseases undergoing allo-HSCT from 10/10 HLA-matched donors. Serum high fibrinogen levels at the time of HSCT (day 0) were significantly associated with cGVHD development in univariate analyses (OR 2.01, p= 0.012) and multivariate analyses (OR 1.003, p= 0.037). There was no significant association between fibrinogen levels and overall survival, disease-free survival and, acute GVHD (p> 0.05). This is the first report demonstrating the association between high fibrinogen levels and increased cGVHD occurrence. Further studies are warranted and may identify the efficacy of fibrinogen as a predictive marker on cGVHD in allo-HSCT recipients. [ABSTRACT FROM AUTHOR]

Objective: Iron deficiency anemia (IDA) affects the quality of life substantially. Recently, different parenteral iron preparations have been used as intravenous iron supplements. The aim of this retrospective study was to assess the efficacy of intravenous ferric carboxymaltose (FCM) and iron sucrose (IS) treatments in patients with IDA. Materials and Methods: The present study included 180 patients treated with intravenous iron at Kayseri Education and Research Hospital. FCM was administered a maximum of two infusions of 500- or 1000-mg iron. Also, IS was 200 mg administered in up to five infusions in 12 days. In all patients, laboratory evaluations were performed before beginning treatment and 4 weeks after treatment. Results: An expected increase in hemoglobin (Hb) and transferrin saturation (TS) levels was observed in the two groups. Also, the median ferritin increase was 54.50 ng/mL in the FCM group, whereas it was 28.05 ng/mL in the IS group (p<0.001). Likewise, post-treatment ferritin levels increased more significantly in the FCM group than in the IS group (58.15 vs. 29.65 ng/mL, respectively) (p<0.001). Conclusion: Our results show high efficacy and good tolerability of both the treatments. Also, FCM has the advantage of allowing more iron to be administered in fewer infusions and rapid correction of ferritin levels in IDA. [ABSTRACT FROM AUTHOR]

Objective: Myelodysplastic syndrome (MDS) represents one of the most frequent and serious hematologic diseases among the elderly. Chromosomal abnormalities have been detected in 23%-78% patients with MDS. We analyzed the cytogenetics of elderly MDS patients in Turkey. Materials and Methods: Data on patients (>65 years old) diagnosed with primary MDS from 2011 to 2013 were retrospectively collected from Erciyes University. Chromosome analysis was performed in 54 patients using conventional karyotyping and fluorescence in situ hybridization (FISH). Results: Of the 54 patients recruited, karyotype abnormalities were found in 8 (15%) of 54 cases, among which, 5 (9%) were Trisomy 8 and 3 (6%) were del 5q. Conclusion: The incidence of chromosomal abnormalities in elderly Turkish MDS patients was lower than that reported in the literature. Although the pathogenesis of MDS is still poorly understood, environmental and biological factors could induce mechanisms that are associated with diverse karyotypes and variable frequencies of chromosomal abnormalities. [ABSTRACT FROM AUTHOR]

Objective: Our aim was to evaluate the contribution of flow cytometry (FC) analysis in patients with a histopathological diagnosis of mantle cell lymphoma (MCL) and to develop a reliable scoring system. Materials and Methods: We assessed the results of FC analysis in peripheral blood or bone marrow samples of 36 patients diagnosed with MCL by histopathological evaluation. Multivariate analysis identified 5 variables associated with MCL: CD23 negativity, CD5/19 common positivity, CD20/22 common bright positivity, FMC7 positivity, and clonal sIg bright positivity. A 5-point scoring system was devised using these parameters. Results: Twenty-five patients (69.4%) scored 5 points, 8 (22.2%) scored 4 points, and 3 (8.4%) scored 3 points. The diagnosis of MCL was confirmed by FC immunophenotyping in 91.6% of the patients. Conclusion: Our results suggest that a quintet diagnostic scoring system, which is considered to be reliable in the diagnosis of MCL, could make a substantial contribution to diagnosis and should be more widely introduced in practice. [ABSTRACT FROM AUTHOR]

Background A variety of apheresis instruments are now available on the market for double dose plateletpheresis. We compared three apheresis devices (Fenwal Amicus, Fresenius COM.TEC and Trima Accel) with regard to processing time, platelet (PLT) yield, collection efficiency (CE) and collection rate (CR). Study Design and Methods The single-needle or double-needle double plateletpheresis procedures of the three instruments were compared in a retrospective, randomized study in 135 donors. Results In the pre-apheresis setting, 45 double plateletpheresis procedures performed with each instrument revealed no significant differences in donor's age, sex, weight, hemoglobin, white blood cell and PLT count between three groups. The blood volume processed to reach a target PLT yield of ≥ 6 × 10 11 was higher in the COM.TEC compared with the Amicus and Trima (4394 vs. 3780 and 3340 ml, respectively; p < 0.001). Also there was a significantly higher median volume of ACD used in collections on the COM.TEC compared with the Amicus and Trima (426 vs. 387 and 329 ml, respectively; p < 0.001). There was a significantly higher median time needed for the procedures on the COM.TEC compared with the Amicus and Trima (66 vs. 62 and 63 min, respectively; p = 0.024). The CE was significantly higher with the Trima compared with the Amicus and COM.TEC (83.57 ± 17.19 vs. 66.71 ± 3.47 and 58.79 ± 5.14%, respectively; p < 0.001). Also, there was a significantly higher product volume on the Trima compared with the Amicus and COM.TEC (395.56 vs. 363.11 and 386.4 ml, respectively; p = 0.008). Additionally, the CR was significantly lower with the COM.TEC compared with the Amicus and Trima (0.092 ± 0.011 vs. 0.099 ± 0.013 and 0.097 ± 0.013 plt × 10 11 /min, respectively; p = 0.039). There was no significant differences in PLT yield between the three groups (p = 0.636). Conclusions Trima single-needle device collected double dose platelets more efficiently than Amicus and COM.TEC double-needle devices. Blood volume processed, ACD-A volume, and median separation time was significantly higher with the COM.TEC. Also, the CR was significantly lower with the COM.TEC. [ABSTRACT FROM AUTHOR]

The use of apheresis equipment to collect blood components has rapidly increased in the past years. A variety of apheresis instruments are now available on the market for double dose plateletpheresis. We evaluated an apheresis instrument, the Trima Accel, with regard to platelet (PLT) yield, collection efficiency (CE), and collection rate (CR) in a retrospective, randomized study in 110 donors. The Trima Accel cell separator efficiently collected double dose platelets with median PLT yields of 3.7 × 10 11 , mean CE of 74.99 ± 14.40% and mean CR of 0.096 ± 0.012 × 10 11 /min. [ABSTRACT FROM AUTHOR]

Abstract: 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 >150×109/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 &y& Elsevier]

Abstract: 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 &y& Elsevier]

Abstract: 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 &y& Elsevier]

Abstract: 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 &y& Elsevier]

Total body irradiation (TBI) combined with chemotherapy is widely used as a pretreatment regimen of bonemarrowtransplantation (BMT) in hematologic disorders. Late complications related to TBI as part of the conditioning regimen for hematopoietic stem cell transplantation have been revealed. Acute transversemyelitis (ATM) is a neurological syndrome characterized by disorder of motor, sensorial, and autonomic nerves, and tracts at medulla spinalis, which is resulted from involvement of spinal cord. In this paper, we presented an ATM attack developed after TBI in a patient with acute lymphoblastic leukemia (ALL) as it is a rarely seen case. [ABSTRACT FROM AUTHOR]

Multiple myeloma is a malignant proliferation of plasma cells that mainly affects bone marrow. Pleural effusions secondary to pleural myelomatous involvement have rarely been reported in the literature. As it is rarely detected, we aimed to report a case in which pleural effusion of a multiple myeloma was confirmed as true myelomatous involvement by flow cytometry method. A 52-years old man presented to our clinic with chest and back pain lasting for 3 months. On the chest radiography, pleural fluid was detected in left hemithorax. Pleural fluid flow cytometry was performed. In the flow cytometry, CD56, CD38 and CD138 found to be positive, while CD19 was negative. True myelomatous pleural effusions are very uncommon, with fewer than 100 cases reported worldwide. Flow cytometry is a potentially useful and simple method for detection of pleural fluid involvement in multiple myeloma. [ABSTRACT FROM AUTHOR]

Platelet (PLT) transfusions play an important role in patients with thrombocytopenia or severely impaired platelet function. Platelet concentrates are prepared from whole-blood donations or by plateletpheresis. In recent years, different instruments have been developed to perform plateletpheresis. We evaluated an apheresis instrument, the Haemonetics MCS ®  + with regard to PLT yield, collection efficiency (CE), and collection rate (CR) in a retrospective, randomized study in 526 donors. In this system, we used leukoreduction filters post collection to obtain leukoreduced products. The Haemonetics MCS ®  + cell separator efficiently collected apheresis platelets with median PLT yields of 3.7 × 10 11 , mean CE of 66.69 ± 13.73% and mean CR of 0.063 ± 0.013 × 10 11 /min. The median blood volume processed was 3290 (2420–4370) ml, and the median volume of acid citrate dextrose-A (ACD-A) used in collections on the device was 385 (196–517) ml. Also, this device allowed the collection of white blood cell (WBC) reduced plateletpheresis with mean 0.07 ± 0.15 × 10 6 WBC content. No serious donor or recipient reactions occurred. [ABSTRACT FROM AUTHOR]

Introduction: Fludarabine, a purine analog, is getting more attention with the increasing use of reduced intensive conditioning regimens in allogeneic hematopoietic stem cell transplantation (allo-HSCT). The side effect of bradycardia was observed in only a few cases reported in the literature. In clinical practice, bradycardia can be asymptomatic or cause syncope and cardiac arrest. This study aimed to evaluate the bradycardia side effect of fludarabine used in the conditioning regimen in allo-HSCT recipients and to increase awareness of this issue. Methods: This retrospective study included 73 patients who received fludarabine in the allo-HSCT conditioning regimen between January 2015 and January 2021. Patients with and without bradycardia were compared regarding demographic data, allo-HSCT characteristics, electrolyte values, fludarabine administration dose and duration, and survival. Univariate and multivariate analyzes were performed to evaluate independent predictors for fludarabine-induced bradycardia. Results: Fludarabine administration doses and days were higher in the bradycardia group, but no statistically significant difference was observed. In the multivariate analysis, age was the only independent predictor of fludarabine-induced bradycardia (odds ratio (OR) 0.93, 95% confidence interval (CI): 0.89–0.98, p = 0.007). The median age in the group with bradycardia was 19 years younger than those without bradycardia (34 (19–49) vs 53 (19–69), p = 0.005). In 11 (84.6%) of the patients who had bradycardia, bradycardia improved with the discontinuation of fludarabine alone, but atropine was administered in 2 (15.4%) patients. Conclusion: Age was the only independent predictor of fludarabine-induced bradycardia; therefore, close heart rate monitoring is recommended during fludarabine administration, especially in younger patients. [ABSTRACT FROM AUTHOR]

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/m2) of CY in 22 patients, and LD (1 g/m2) 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. [ABSTRACT FROM AUTHOR]

Niemann-Pick disease is a rare lipid storage disorder with autosomal recessive inheritance, which is characterised by the accumulation of sphingomyelin and other sphingolipids in macrophages. Kartagener's Syndrome is a syndrome with autosomal recessive inheritance consisting of chronic paranasal sinusitis, situs inversus, and bronchiectasis. Here we reported a case having Kartagener's Syndrome with concomitant Niemann- Pick disease, as such a case has not been reported in the literature. [ABSTRACT FROM AUTHOR]

Abstract: Ataxia-telangiectasia (AT) is a hereditary disorder characterized by progressive neurological dysfunction, oculocutaneous telangiectasia, immunodeficiency, cancer susceptibility, and radiation sensitivity. Pediatric patients may develop acute lymphoblastic leukemia (ALL). However development of ALL in an adult patient with AT is a rare occurrence. Here we report such a patient who presented with hyperleukocytosis and were treated with leukapheresis. A 25years old male patient, who were diagnosed with AT and mental retardation, was admitted to the emergency department due to fatigue, nausea and headache. On admission he had a moderate general condition and was fully cooperated. His white blood cell (WBC) count were 466×109/l. Blastic cells were observed in peripheral blood smear. Flow cytometry (FC) of peripheral blood showed T-ALL. Two sessions of large volume leukapheresis was performed. Symptoms due to hyperleukocytosis markedly improved after leukapheresis. Patients with AT should be closely monitored due to risk of malignancy. Leukapheresis may improve the prognosis of high risk ALL patients presenting with hyperleukocytosis. [Copyright &y& Elsevier]

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. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 63-year-old male who was diagnosed with acute promyelocytic leukemia after evaluation of peripheral blood smear and bone marrow. The patient developed fever, infraorbital edema, and yellow-black discoloration of the upper palate after undergoing chemotherapy which included cytarabine and idarubicin. The patient died on 30th day of hospitalization after surgical debridement. It also discusses mucormycosis.

Purpose: Clofarabine is a second-generation purine analog that inhibits DNA synthesis. It is used as an effective new agent in relapsed refractory acute leukemia. We aimed to report our single center experience about CLOVE protocol as a clofarabine-based regimen in patients with relapsed or refractory T-cell acute lymphoblastic leukemia. Materials and Methods: Thirteen patients with relapsed or resistant T-cell acute lymphoblastic leukemia were included in this study. Patients were administered clofarabine (40 mg/m²/day), etoposide (100 mg/m²/day), and cyclophosphamide (440 mg/m²/day) (5 days chemotherapy). The patients' data were reviewed retrospectively, the demographic and clinical characteristics of patients were recorded. Results: The patients' median age was 47, and 77% (n:10) of them were male. The median number of chemotherapy regimens they received before clofarabine was 3. Of the patients, 54% (n=7) presented with relapse, and 46% (n=6) with refractory disease. Hematological side effects were observed in all patients during treatment, and 4 (31%) patients were complicated by febrile neutropenia. Other side effects were hepatotoxicity 39%, skin reaction 2%, nausea 54%, and mucositis 31%. Three (23%) patients were unresponsive to treatment. A response was obtained in 10 (77%) patients. Allogeneic stem cell transplantation was performed in 4 of 5 patients with complete response. The median follow-up time after CLOVE was 2.3 (0.69-26.02) months. The median estimated survival time was 21.04 ± 3.88 (95%CI: 13.43 -28.64) months. Overall survival was 85.7% at three months and 57.1% at one year. Three patients were alive at the end of our study. Conclusion: The combination of clofarabine, etoposide, and cyclophosphamide (CLOVE) appears to be successful in achieving a response in relapsing or resistant acute leukemia. However, more effective regimens are still needed. [ABSTRACT FROM AUTHOR]

Objectives and Aim In this study, we aimed to compare the potency of different G-CSF agents including original filgrastim (Neupogen®), biosimilar filgrastim (Leucostim®) and Lenograstim (Granocyte®) on CD34 + cell mobilization in patients that underwent allogeneic hematopoietic stem cell transplantation (alloHSCT). Patients and Methods The data of 243 donors for alloHSCT recipients diagnosed with mostly acute leukemia and myelodsyplastic syndromes (MDS) were analyzed, retrospectively. Data for stem cell mobilization have been recorded from patients' files. Donors who received Filgrastim (Neupogen®, Group I), biosimilar Filgrastim (Leucostim®, Group II) and Lenograstim (Granocyte®, Group III) were analyzed for total CD34 + cell count at the end of mobilization procedures. Results A total of 243 donors and patients for alloHSCT were analyzed retrospectively. The diagnosis of the patients were; acute myeloid leukemia (AML) (110 patients, 45.2%), acute lymphoid leukemia (ALL) (61 patients, 25.1%), aplastic anemia (AA) (38 patients, 15.6%), lymphomas (14 patients, 5.7%) and others (20 patients, 8.4%). The median number of total collected PB CD34 + cells (×10 6 /kg) was 7.12 (min–max: 5.38–7.90) in the Neupogen® group, 7.27 (min–max: 6.79–7.55) in the Leucostim® group and 7.15 (min–max: 5.34–7.58) in the Granocyte® group. There was no statistically significant difference among groups in terms of total collected PB CD34 + cells (p = 0.919). The median doses of G-CSF agents (µg/kg/day) in PBSC collection in Neupogen® group was; 11.00 (10.00–12.00) in Leucostim® group10.35 (min–max: 10.00–11.10) and in Granocyte® group11.00 (min–max: 10.00–11.00). There was no statistical significance among groups (p = 0.215). Conclusion Biosimilar filgrastim (Leucostim®) was found comparable to original Filgrastim (Neupogen®) and Lenograstim (Granocyte®) for PBSC mobilization in donors of the patients that underwent alloHSCT. [ABSTRACT FROM AUTHOR]

Objective: We aimed to determine the coronavirus disease 2019 (COVID‐19) fear state in haematopoietic stem cell transplant patients and to examine its relationship with quality of life. Methods: In this prospective study, 64 patients who underwent HSCT during the pandemic were included. The COVID‐19 fear situation was evaluated with the Fear of COVID‐19 Scale (FCV‐19S). Quality of life was evaluated with the European Organisation for Quality of Life Research and Treatment Core Questionnaire (EORTC QLQ‐C30) (Version 3). Results: The median FCV‐19S score was 16.5 (12.0–22.0). The FCV‐19S score was significantly higher in urban residents than rural residents. The general health score was 59.64 ± 20.04. The strongest positive correlation between fear level and life quality was found in emotional function. A weak, significant, positive correlation was observed between role function, nausea–vomiting, pain, appetite loss and fear level. Conclusion: FCV‐19S is a quick, safe and valid tool that can be used to determine the COVID‐19 fear level in vulnerable patient groups such as HSCT patients and to direct them to the necessary psycho‐oncological support. [ABSTRACT FROM AUTHOR]

Diffuse large B-cell lymphoma(DLBCL) is the prototype of aggressive lymphomas. The abnormalities such as translocation, deletion, duplication are stated to affect the progress and the prognosis of the disease. This study investigates the presence and impact on the prognosis of K-ras mutation and C-myc overexpression in patients with DLBCL. Twenty eight patients diagnosed with DLBCL and 29 healthy controls, whose diagnostic lymph node biopsy was reactive, were enrolled in this study. Paraffin blocks were analyzed for K-ras mutations and C-myc expressions. The effects of C-myc and K-ras on overall and disease-free survival were evaluated. Of the 28 patients, three (10.7%) K-ras mutations were detected. K-ras mutation (+) cases have an average survival time of 28 months, while those without the mutation were 49 months. The average duration of disease-free survival was 17.6 months in patients with K-ras mutation, while it has been identified as 45 months in patients without mutations (p= 0.072). C-myc overexpression in 17 patients(61%) was detected. Median overall survival was 33 months in male patients, while it was 65 months in female patients (p= 0.006). The median disease-free survival time was 23 months in male patients, although 64 months in female patients (p= 0.003). Complete remission rate was 30% in C-myc overexpression group, while 69% in remaining group with the cut-off 2.48 (p= 0.02). The presence of K-ras and C-myc overexpression in patients with DLBCL does not affect overall and disease-free survival. C-myc overexpression harms complete remission. Gender was found to be influential on the overall and disease-free survival; meanwhile, the female gender was a better prognosis. [ABSTRACT FROM AUTHOR]

Aim: Primary mediastinal large B-cell lymphoma (PMBCL) is a type of lymphoma that forms approximately 3 % of non-Hodgkin lymphomas that often encounter with mass. The aim of this study was to present the epidemiological characteristics, response rates of the treatment and the survival of PMBCL patients in our single center. Materials and Methods: Patient demographics, treatment regimens, survival rates of PMBCL patients were retrospectively analyzed. Results: There are 15 patients in our study. Most of the patients were female (n:9, 60%). The median age at the time of diagnosis was 35.4. Nine patients applied with a bulky lesion in the mediastinum. Most of the patients have been treated with DA-EPOCH-R (dose-adjusted etoposide, doxorubicin, cyclophosphamide, vincristine and prednisone with rituximab) (n:13, 87%) and 2 (n:2, 13%) patients have been treated with R-CHOP (doxorubicin, cyclophosphamide, vincristine and prednisone with rituximab) regimens for 6 cycles. Eight patients (53.3%) received involved-field radiotherapy on the mediastinum. After the first-row chemotherapy, total remission rate was 93.3%. Thirteen (87%) of the patients were still in remission and alive. The median follow-up time in our study was 43 months (13 -81). Mean disease-free survival was 67.43 months and overall survival was 72.87 months. The overall and disease-free survival rate was 86.7 % and 80%. Conclusions: In our study, most patients responded to the treatment and are still being followed in remission. [ABSTRACT FROM AUTHOR]

Objective: 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 dampen T lymphocyte attack. The checkpoint inhibition by tumor cells via the PD-1 pathway suppress the antitumor immune response. The role of PD-1 pathway has been extensively investigated in non-hematologic malignancies, however, the exact role of this pathway is not established in hematologic disorders. So, we aimed to demostrate the PD-1 and PD-L2 expresion rate of various lymphoma subtypes, and to evaluate whether PD-1 and PD-L2 expresion have impact on prognosis. Method: For this purpose, pre-treatment lymph node biopsy specimens of 92 patients [25 Hodgkin lymphoma (HL) and 67 non-Hodgkin lymphoma (NHL)] have been stained with monoclonal antibody immunstains of PD-1 and PD-L2. Results: The overall expression rate of PD-1 was 76% and 82.1% in patients with HL and NHL, respectively. PD-L2 expression rate was weak in both HL and NHL cases. Since we have evaluated whether there is a correlation between immunohistochemistry (IHC) results and survival of patients with HL and NHL, we couldn't demonstrate a meaningful evidence that these markers have an impact on prognosis. Conclusions: We conclude that the role of PD-1 pathway can be demonstrated by IHC. If IHC markers might be standardized in the future, especially a cutoff that defines a clinically significant positive and predictive value, may help identifying patients more likely to benefit from anti-PD-1 therapies. [ABSTRACT FROM AUTHOR]

Objective: The reactivation rate of dormant hepatitis B virus (HBV) in the liver is between 4.1% and 41.5% in immune-compromised patients. The disappearance of hepatitis B surface antibody (anti-HBs) can be an alarm for hepatitis B reactivation. In this study, the changes in serological status, reactivation rates, and antiviral prophylaxis rates were evaluated. Materials and Methods: A retrospective study was conducted involving patients who were followed up at Erciyes University HSCT Center between January 2018 and July 2019. The demographic data, type of hematological disease, pre- and post-transplant status of HBV, presence of antiviral prophylaxis, and frequency of hepatitis B flare were evaluated. Results: One hundred and seven patients were included in this study. The median follow-up duration was 18 months. New chemotherapy protocols were initiated in 36 patients due to progression and in 23 patients with a diagnosis of graft-versushost disease. Anti-HBs levels decreased in 60% of the patients, and anti-HBs levels decreased to below the protective level in 13% of the patients. Among the 107 patients, 38 had resolved hepatitis B infection before transplantation, and 20 and four of 18 patients (22%) who did not receive antiviral prophylaxis developed HBV seroconversion and hepatitis B flare. The median levels of anti-HBs titers after transplantation were 167 IU/L and 15 IU/L in groups that received and did not receive antiviral prophylaxis, respectively (p=0.028). Conclusion: Antiviral prophylaxis should be administered in patients positive for hepatitis B core antibody before hematopoietic stem-cell transplantation. Measuring HBV serological parameters at regular intervals is essential in the high-risk group. [ABSTRACT FROM AUTHOR]

Factors such as age, underlying hematological disease, chemotherapy and radiotherapy used, and bone marrow infiltration may cause mobilization failure. Several preclinical observed that diabetes mellitus (DM) leads to profound remodeling of the hematopoietic stem cell (HSC) niche, resulting in the impaired release of HSCs. We aim to examine the effect of DM on HSC mobilization and to investigate whether there is a relationship between complications developing in the DM process and drugs used to treat DM and mobilization failure. In Erciyes University Bone Marrow Transplantation Unit, 218 patients who underwent apheresis for stem cell mobilization between 2011 and 2021 were evaluated retrospectively. One hundred and nine patients had a diagnosis of DM, and 109 did not. Mobilization failure developed in 17 (15.6 %) of the patients in the DM group, while it developed in 7 (6.4 %) patients in the non-DM group (p = 0.03). CD34+ stem cell count was 8.05 (1.3–30.2) × 106/kg in the DM group, while it was 8.2 (1.7–37.3) × 106/kg in the other group (p = 0.55). There was no statistically significant relationship between glucose and hemoglobin A1c levels and the amount of CD34+ cells (p = 0.83 and p = 0.14, respectively). Using sulfonylurea was the only independent predictor of mobilization failure (OR 5.75, 95 % CI: 1.38–24.05, p = 0.02). DM should be considered a risk factor for mobilization failure. Further research is needed fully to understand the mechanisms underlying the mobilization failure effects of sulfonylureas and to develop strategies to improve stem cell mobilization in diabetic patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: Multiple Myeloma is an incurable fatal disease accounts for 1% of all the cancer and 10% of the haematological malignancies. Generally, it is seen in elderly population. The aim of the present study was to present our experience in 106 Multiple Myeloma patients aged 65 years or older and treated with different regimens during the 5 years term preceding the study. Method: In order to analyse the clinical characteristics and therapeutic outcomes of the Multiple Myeloma patients over 65 years, data were gathered retrospectively from 5 different centres in Turkey. Results: A total of 105 elderly Multiple Myeloma patients (aged 65 years or older) composed of 51 males and 54 females were evaluated retrospectively. Vincristine, Adriamisin, Dexamethasone (VAD), Bortezomib, Cyclophosphamide, Dexamethasone (VCD), Melphalan, Prednisolone (MP) Melphalan, Prednisolone, Thalidomide (MPT) regimens were given as the first line. 29 patients yielded complete responses and 18 of these patients underwent autologous stem cell transplantation. In 46 patients out of those not yielding a full response were treated with bortezomib, lenalidomide and thalidomide based second line treatments. 29 of those treated with a second line treatment yielded a complete response and 20 of these patients underwent autologous stem cell transplantation. 16 patients not responding to the first and second line treatments were treated with a third line treatment using a lenalidomide based (n=10) and thalidomide based (n=6) regimens. 5 patients were subjected to autologous stem cell transplantation due to remission status. Conclusions: An optimal treatment approach should make a productive, safe and high quality life possible while the aim should be to ensure a disease-free survival and prolong the general survival along with obtaining a full response. [ABSTRACT FROM AUTHOR]

Abstract: 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 122days (min–max:91–225). The iron chelating treatment significantly reduced serum ferritin levels administered at a dosage of 20–30mg/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 &y& Elsevier]

Abstract: Aim: We aimed to investigate the change in the number of stem cells and white cells in the early period following blood donation. Patients and Method: 22 male (71%) and 9 female (29%), 31 volunteers in total were included in the study. 450ml of whole blood were collected from each of the volunteers for the donation. Complete blood counts were performed on the volunteers before and at 6 and 24h after the donation and CD34+ cell counts per ml of peripheral blood were measured by flow cytometry technique. Results: There was a statistically significant increase in the number of CD34+ cells in the peripheral blood at 6h following blood donation (p <0.001). At 24h, however, there was a statistically significant decrease in the number of CD34+ cells, compared to 6h (p <0.001). There was a statistically significant increase in the number of leukocytes in the peripheral blood at 6h following blood donation (p <0.001). At 24h, there was a decrease in the number of leukocytes, which was statistically significant compared to 6h (p <0.001). When the difference in CD34+ cell and leukocytes counts before blood donation and at 24h after blood donation were compared, the results were not statistically significant. Conclusion: As the result of this study, a transient increase in the number of CD34+ cells in the peripheral blood after blood donation was demonstrated, with a decline in CD34+ cell counts back to levels prior to donation at 24h. [Copyright &y& Elsevier]

Objectives and aim: Patients affected by hematological malignancies can often benefit from high dose chemotherapy followed by peripheral blood stem cells (PBSCs) transplantation. Different strategies have been used to mobilize an adequate number of PBSC, including granulocyte colony-stimulating factor (G-CSF) alone or chemotherapy plus G-CSF. In this study, we aimed to compare the efficacy profile of different G-CSF agents including filgrastim (Neupogen®), biosimilar filgrastim (Leucostim®) and Lenograstim (Granocyte®) on CD34+ mobilization in patients who underwent autologous hematopoietic stem cell transplantation (autoHSCT). Materials and methods: We retrospectively analysed data of patients who underwent autoHSCT diagnosed with multiple myeloma (MM), Hodgkin Lymphoma (HL), non-Hodgkin Lymphoma (NHL) and others. Data for stem cell mobilization has been obtained from patients’ files. Patients who received Filgrastim (Neupogen®), biosimilar Filgrastim (Leucostim®, Group) and Lenograstim (Granocyte®) were evaluated mainly for total CD34+ cell count at the end of mobilization procedure. Results: A total of 96 patients who underwent autoHSCT were retrospectively analyzed. 27 (28.2%) of the patients were female, and 69 (71.8%) were male. The diagnosis of the patients were; multiple myeloma (39 patients, 40.6%), Hodgkin Lyphoma (23 patients, 23.9%), non-Hodgkin lymphoma (16 patients, 16.6%), and others (18 patients, 18.9%). The median number of leukapheresis cycle necessary to harvest a minimal count of 3×106 CD34+/kg was 2 in Neupogen® (min–max: 1–4) and Granocyte® (min–max: 1–3) groups and 1 (min–max: 1–2) in Leucostim® group. The median doses of G-CSF agents (μg/kg/day) in PBSC collection procedure were; 10.00 (min–max: 7.00–12.00) in the Neupogen® group, 8.00 (min–max: 7.25–9.00) in the Leucostim® group and 8.50 (6.00–9.50) in the Granocyte® group. There was no statistical significance among groups (p =0.067). The number of total collected PB CD34+ cells (×106/kg) was 7.64 (min–max: 4.09–13.86) in the Neupogen® group, 13.43 (min–max: 8.15–23.38) in the Leucostim® group and 5.45 (min–max: 4.28–9.40) in the Granocyte® group. The data showed that patients in the leucostim group had significantly higher PB CD34+ cells compared to patients in the Granocyte® group (p =0.013). Conclusion: Leucostim® was comparable to Neupogen® for PBSC mobilization in patients who underwent autoHSCT. [ABSTRACT FROM AUTHOR]

Abstract: Invasive fungal pneumonia (IFP) has become increasingly common in patients that previously underwent alloHSCT. The aim of this study was to determine the role of hyperferritinemia, via iron overload in invasive fungal pneumonia in patients that underwent alloHSCT. Medical records of 73 patients with pneumonia that underwent alloHSCT were studied retrospectively, whereby a pre-transplantation serum ferritin level measured up to 100days prior to transplantation of patients with invasive fungal pneumonia (IFP) and non-fungal pneumonia (non-IFP) was compared. Patient records revealed 35 and 38 cases of IFP and non-IFP, respectively. In risk evaluation for IFP, age, gender, HLA status, conditioning regimen, smoking history, and underlying disease were not significantly different among groups (p >0.05). However, performance status (Karnofsky) was significantly lower in patients with IFP (p <0.05). The median ferritin levels were 1705ng/ml (41–7198) in the IFP group and 845ng/ml (18–7099) in non-IFP group and the difference was found statistically significant (p =0.001). Elevated pretransplant serum ferritin level is associated with IFP in patients that underwent alloHSCT, in particular when values exceed 1550ng/ml. [Copyright &y& Elsevier]

A 59-year-old female patient was admitted to the emergency service with complaints of hematemesis and melena for the last few days. In laboratory tests, the platelet count was found to be 6 × 109/L. Intravenous or oral corticosteroid treatment was thought to be given for ITP but disclaimed due to upper GIS bleeding. On the 5th day of treatment, Brucella melitensis was isolated from blood culture before the results of Wright tube agglutination tests were reported positive as 1 : 80. On the second day of the antibrucellosis treatment, the thrombocyte count was raised from 6000/mm³ to 110000/mm³, and on the 3rd day to 225000/mm³. [ABSTRACT FROM AUTHOR]

Aims: Despite the clinical importance of the leukemic transformation of chronic myeloproliferative neoplasms (MPNs), very little is known about markers that predict leukemic transformation. We studied WT1 expression in 37 MPN patients diagnosed as bcr-abl negative and JAK2 (V617F) positive with a molecular genetic test, and 23 healthy controls. Results: WT1 expression is higher in MPN patients compared with normal controls ( p=0.002). According to the WT1 expression levels, patients were divided into two groups: high (≥0.205) and low (0-0.205) WT1 expression. Two out of six patients with a high WT1 expression level transformed to myelodysplastic syndrome at a 42- and 46-month follow-up, respectively. Conclusions: Our results suggest that the overexpression of WT1 may play an important role in the leukemic transformation of MPNs. [ABSTRACT FROM AUTHOR]