Effect of Blood Groups on Clinical Presentations and Treatment Outcomes in Immune Thrombotic Thrombocytopenic Purpura Patients with Severe ADAMTS13 Deficiency: A Multi-Center Experience.

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by accumulation of ultra-large von Willebrand factor (vWF) due to the significantly reduced activity ADAMTS13. Limited studies have been published examining the blood group as an epidemiological factor that can contribute to development of TTP. It has been suggested that due to low vWF levels, the distribution of the "O" blood group among TTP patients may be lower than anticipated compared to the blood group distribution rates in the normal population. The aim of this study was to explore the relationship between blood groups and the clinical outcome of immune TTP (iTTP). Methods: Thirty patients with iTTP with severe ADAMTS13 deficiency were enrolled. Data collection commenced in January 2011 and was completed by June 2020. It was analyzed whether there was a difference between the blood groups in terms of frequency of iTTP, response to treatment, frequency of relapse, and clinical and laboratory results. Results and Conclusions: The distribution of group "A" among patients with iTTP was higher than expected. Although not statistically significant, patients with blood group "O" required more TPE for the treatment and relapse rate was statistically higher than other blood groups. Mortality rate in all patients was 6.7%. Although blood group "A" is a risk factor for iTTP, the frequency of relapse is higher in the blood group "O."

Brentuximab vedotin consolidation therapy after autologous stem-cell transplantation in patients with high-risk Hodgkin lymphoma: Multicenter retrospective study.

The AETHERA trial reported an increased progression-free survival (PFS) when brentuximab vedotin (BV) was used as maintenance therapy in high-risk Hodgkin lymphoma (HL) after autologous stem cell transplantation (ASCT). Thus, we aimed to determine the impact and safety of BV as maintenance after ASCT in real-world patients. Seventy-five patients with relapsed/refractory HL started on BV consolidation therapy after ASCT due to high risk of relapse, between January 2016 and July 2019, from 25 institutions, were included in the study. The median follow-up time was 26 months. The most common high-risk features were primary refractory or relapsed disease <12 months (n = 61), lack of complete response (CR) to the last salvage regimen (n = 51), and having had at least two salvage regimens (n = 29). At the time of analysis, 42 patients completed consolidation courses, and BV was discontinued in 33 patients. Fifty patients had an ongoing response (CR in 41, PR in 6, and SD in 3 patients), 25 had progressed. Ten died in the follow-up, eight with progressive disease and two due to infection while in CR. The 2-year PFS and OS rates were 67.75% (95% confidence interval [CI]: 0.55-0.77) and 87.61% (95% CI: 0.76-0.94), respectively. Seventeen patients (23%) received BV in the pre-ASCT treatment lines, and there was no survival difference between the BV-naïve and BV-exposed groups. The most common adverse events were neutropenia (27%) and peripheral neuropathy (21%). Sixteen patients (21.3%) experienced grade 3 or 4 toxicity. BV was discontinued due to adverse event in 12 patients. Consolidation with BV after ASCT can achieve a 2-year PFS of 67.75% (95% CI: 0.55-0.75) with an acceptable toxicity profile.

Classical Hodgkin lymphoma-like post-transplant lymphoproliferative disease after allogeneic stem cell transplantation for primary myelofibrosis is successfully treated with nivolumab: A case report.

INTRODUCTION: Post-transplant lymphoproliferative disease (PTLD), a lymphoid proliferation observed after the solid organ transplantation or allogeneic stem cell transplant, is an important and mortal complication that can occur during the post-transplant period. Classical Hodgkin lymphoma-like PTLD is the least form of PTLD. We are presenting an adult case of classical Hodgkin lymphoma-like PTLD which was successfully treated with nivolumab. CASE REPORT: A 31-year-old female was diagnosed with primary myelofibrosis and we performed allogeneic stem cell transplantation from her HLA fully matched brother in 2015. Two years after transplant, classical Hodgkin lymphoma-like PTLD was diagnosed. The patient was resistant to six cycles of ABVD chemotherapy and four cycles of brentuximab vedotin. MANAGEMENT AND OUTCOME: After the failure of ABVD and brentuximab vedotin, we started nivolumab therapy at a dose of 3 mg/kg every 2 weeks. After six cycles, we achieved a PET negative complete remission. After 10 cycles of nivolumab, the patient is still followed with a complete remission. Still, there is no evidence of acute or chronic GvHD, and therefore no need for immunosuppressive treatment. No auto-immune complication was observed. It is planned to give nivolumab treatment to the patient until the progression. DISCUSSION: Our case has depicted that the classical Hodgkin lymphoma type PTLD may be resistant to the conventional treatments and anti-CD30 brentuximab vedotine. In such cases, nivolumab may be an effective and worth assessing agent in terms of both activity and safety profile.

Comparison of allogenic stem cell transplantations performed with frozen or fresh stem cell products with regard to GVHD and mortality.

PURPOSE: Stem cells are collected from donors and infused to the recipient in allogenic peripheral stem cell transplantations. The use of frozen stem cells can promote donor compatibility, and overcoming possible problems due to insufficient stem cell mobilization will also be easier. Nevertheless, studies about the use of frozen peripheral stem cells in allogenic transplantation are extremely rare. In this study, we aimed to compare the clinical outcomes of allogenic stem cell transplants from frozen or fresh stem cell products. MATERIALS AND METHODS: This retrospective analysis was conducted between April 2004 and September 2018 in the bone marrow transplantation unit of Ankara Numune Training and Research Hospital. Clinical data of patients who received allogenic peripheral stem cell transplantations from fully matched sibling donors were compared for 42 fresh and 30 frozen stem cell transplants. RESULTS: While the platelet engraftment period, febrile neutropenia period, hospitalization period, and 100-day mortality rates did not show any differences, the neutrophil engraftment period was longer in the frozen group (mean: 14 days vs. 16 days, p = 0.006). Acute and chronic graftversus-host disease (GVHD) rates were similar in both groups; however, the rate of grade 3 or4 chronic liver GVHD was slightly higher in transplants performed with fresh stem cells compared to the frozen group (p = 0.046). Overall survival was similar between the groups (p = 0.700). CONCLUSION: The use of frozen peripheral stem cells in allogenic stem cell transplantation may be a reasonable option that can be applied without causing a significant change in clinical results.

CD38 Expression and Variation as a Prognostic Factor Chronic Lymphocytic Leukemia.

BACKGROUND: In this study, we aimed to determine a cutoff level for CD38 that would aid us in identifying chronic lymphocytic leukemia patients in need of early therapy and predicting patients at sufficiently low risk who would likely exhibit a rapid improvement; we also aimed to find out if CD38 expression would show variability during disease course and determine the extent of CD38 expression. METHODS: 124 patients were diagnosed with CLL. CD38 and ZAP-70 expression levels were measured with four color flowcytometry. Time from diagnosis to initial therapy was calculated for all patients. CD38 expression was studied for a second time during follow-up in 50 patients. RESULTS: For cutoff levels of 7%, 20%, and 30%, CD38 expressions were 61.3%, 25%, and 24.2%, respectively. At all three cutoff levels there were significant correlations with all parameters except age between CD38+ vs. CD38- groups (p < 0.001). The comparative rates of starting therapy for cutoff levels of 7%, 20%, and 30% in CD38+ and CD38- groups were 77.5% vs. 6.25%; 100% vs. 30.7%, and 100% vs. 31.5%, respectively (p < 0.001). Multiple Cox Proportional Hazards Regression analysis: for a cutoff level of 7%, survival was affected by STAGE, ZAP70, and CD38. CONCLUSIONS: A CD38 cutoff level of 7% determined by standardized laboratory techniques is an important prognostic marker. However, the number and frequency of repeat measurements of CD38 expression, and cutoff level of CD38 expression that significantly predict disease prognosis should be further determined by future cohort studies.

A rare case of essential thrombocythemia with pseudo-hyperkalemia.

Essential thrombocythemia (ET) is a rare clonal stem cell disorder that affects the production of platelets in the bone marrow. This condition causes an overproduction of platelets, which can lead to blood clots and other complications. Potassium, on the other hand, is an essential mineral that plays a vital role in various bodily functions, including nerve impulses and muscle contractions. Here, in this case report, we investigated a case of pseudo-hyperkalemia caused by essential thrombocythemia in a 77-year-old woman with very high platelet counts. Moreover, this case report, which has no similar examples in the literature review, is important for clinicians.

High CRP-albumin ratio is associated high thrombus burden in patients with newly diagnosed STEMI.

In patients undergoing primary percutaneous coronary intervention (pPCI) due to ST-segment elevation myocardial infarction (STEMI), an increased intracoronary thrombus burden is a strong predictive factor for adverse cardiovascular events. The C-reactive protein (CRP)-serum albumin (SA) ratio (CAR), used as an inflammatory marker, is closely associated with thrombogenicity. In this study, we investigated the relationship between coronary thrombus burden and CAR in patients undergoing pPCI due to newly diagnosed STEMI. A total of 216 patients who underwent pPCI due to STEMI were retrospectively included for the study. Angiographic thrombus burden was assessed according to thrombolysis in myocardial infarction (TIMI) grading, and those with grade 1, 2, 3 were classified as low thrombus burden (n = 120) and those with grade 4, 5 were classified as high thrombus burden (HTB) (n = 96). CAR was calculated as the ratio of CRP to SA. The average age of the patients was 60 ± 9.8, and the male ratio was 61.1. Compared to the LTB group, the HTB group had higher CAR, age, SYNTAX score, baseline cTnT, peak cTnT, CRP, glucose, WBC, and NLR while the LVEF and SA levels were lower (P < .05). Spearman's correlation analysis revealed a significant correlation between thrombus burden and CAR. The multivariable logistic regression analysis revealed that CAR (odds ratio: 10.206; 95% confidence interval: 2.987-34.872, P < .001) was a independent risk factor for HTB. According to the receiver operating characteristic (ROC) analysis, when the cutoff value for CAR was taken as ≥1.105 CAR could predict HTB with a sensitivity of 70.8% and specificity of 67.7%. Our data indicate that CAR an independent risk factor for thrombus burden.

A novel differential diagnosis algorithm for chronic lymphocytic leukemia using immunophenotyping with flow cytometry.

INTRODUCTION: The availability of a clinical decision algorithm for diagnosis of chronic lymphocytic leukemia (CLL) may greatly contribute to the diagnosis of CLL, particularly in cases with ambiguous immunophenotypes. Herein we propose a novel differential diagnosis algorithm for the CLL diagnosis using immunophenotyping with flow cytometry. METHODS: The hierarchical logistic regression model (Backward LR) was used to build a predictive algorithm for the diagnosis of CLL, differentiated from other lymphoproliferative disorders (LPDs). RESULTS: A total of 302 patients, of whom 220 (72.8%) had CLL and 82 (27.2%), B-cell lymphoproliferative disorders other than CLL, were included in the study. The Backward LR model comprised the variables CD5, CD43, CD81, ROR1, CD23, CD79b, FMC7, sIg and CD200 in the model development process. The weak expression of CD81 and increased intensity of expression in markers CD5, CD23 and CD200 increased the probability of CLL diagnosis, (p < 0.05). The odd ratio for CD5, C23, CD200 and CD81 was 1.088 (1.050 - 1.126), 1.044 (1.012 - 1.077), 1.039 (1.007 - 1.072) and 0.946 (0.921 - 0.970) [95% C.I.], respectively. Our model provided a novel diagnostic algorithm with 95.27% of sensitivity and 91.46% of specificity. The model prediction for 97.3% (214) of 220 patients diagnosed with CLL, was CLL and for 91.5% (75) of 82 patients diagnosed with an LPD other than CLL, was others. The cases were correctly classified as CLL and others with a 95.7% correctness rate. CONCLUSIONS: Our model highlighting 4 markers (CD81, CD5, CD23 and CD200) provided high sensitivity and specificity in the CLL diagnosis and in distinguishing of CLL among other LPDs.

Wet milling of buckwheat cultivars and some quality properties of the fractions.

The hulled buckwheat cultivars (Aktas cv. and Günes cv.) were wet-milled, and then some chemical, yields, colour, functional properties, phenolic compound, antioxidant activity, and pasting, thermal and retrogradation properties of starches were investigated and compared with the wholegrain buckwheat flour (with hull) and buckwheat groat flour (without hull) of the same cultivars. Sodium dodecyl sulphate polyacrylamide gel electrophoresis patterns of flours and protein fractions were examined under reducing and non-reducing conditions. The hull, germ+dietary fibre, protein and starch fractions were collected. The total recovery for Aktas cv. and Günes cv. cultivars were 98.1% and 96.1%; total starch yields were 51.6% and 49.7%; pasting temperatures of the starches were found as 83.7 and 85.7°C; and final viscosities of starches were determined as 3.5 and 3.4 Pa·s, respectively. The resistant starch contents of starch fractions of Aktas cv. ve Günes cv. were found as 3.28% and 3.62%, respectively. The highest total phenolic compound contents were detected with dimethyl sulphoxide extraction in the germ+dietary fibre fractions. The highest 2,2-diphenyl-1-picrylhydrazyl radical scavenging activity and trolox equivalent antioxidant capacity were found in the hull fraction (as 81.7%) and germ+dietary fibre fraction (as 11.8 mmol/kg) of Aktas cv. cultivar.

Evaluation of Children with Acute Rheumatic Fever: A Single-Center Experience.

OBJECTIVE: The epidemiological characteristics, risk factors, complications, recurrence status, clinical and laboratory features, and treatment methods of the patients who admitted to our Pediatric Cardiology Outpatient Clinic with a pre-diagnosis of acute rheumatic fever (ARF) were evaluated. MATERIALS AND METHODS: The data of 166 patients who admitted with a pre-diagnosis of ARF and were diagnosed with ARF, and the data of 51 patients who were not diagnosed with ARF, were retrospectively analyzed. RESULTS: The patients with ARF were between the ages of 5 and 18. Most of the patients with ARF attack admitted in December (15.6%), January (13.8%), and February (13.2%). The most common complaints of the patients diagnosed with ARF were isolated joint pain and/or swelling, at 50.6%. While 91.5% of the patients were diagnosed for the first time, 8.5% had ARF recurrence. It was seen that the most common major criterion was carditis (94.6%). The severity of valve regurgitation and the rates of monoarthritis were significantly higher in patients with recurrence (P < .05). Non-compliance with prophylaxis was observed in 10 (71.4%) of 14 patients with recurrence, and in 43 (28.2%) of 152 patients without recurrence. Anti-streptolysin O was lower (P = .021) and alanine transaminase (ALT) was higher (P = .019) in the recurrence group. CONCLUSION: Our study showed that in patients with a pre-diagnosis of ARF, a differential diagnosis should be made with other diseases. Especially in patients with joint complaints as the only major symptom, a differential diagnosis should be made. ARF recurrence is associated with non-compliance with prophylaxis, and both the severity of valve regurgitation and monoarthritis rates are higher in patients who develop recurrence. Alanine aminotransferase is significantly higher in patients with ARF recurrence.

Comparison of Mitoxantrone-Melphalan and BEAM Conditioning Regimens in Patients with Lymphoma.

OBJECTIVE/BACKGROUND: Lymphoma is seen as a highly treatable and curable malignancy with aggressive treatment methods. Efficacy is often limited by toxicity and many patients need alternative treatment strategies as they cannot tolerate existing high cytotoxic approaches. Our aim is to compare BEAM [carmustine (BCNU), etoposide, cytarabine (ARA-C, cytosine arabinoside), and melphalan] and mitoxantrone-melphalan (Mx-Mel) regimens utilized in our patients with a diagnosis of lymphoma who underwent autologous stem cell transplantation (ASCT), and to demonstrate that the Mx-Mel regimen has similar but less toxic results than the BEAM regimen we have been using frequently as standard conditioning regimen. METHODS: A total of 101 patients with lymphoma who underwent ASCT were included in our study. The BEAM regimen included BCNU, etoposide, ARA-C, and melphalan. The Mx-Mel regimen included mitoxantrone and melphalan. RESULTS: Of 101 patients included in the study, 60 (59.4%) received BEAM and 41 (40.6%) received Mx-Mel (40.6%) conditioning regimen. The median time to neutrophil engraftment was 10 (range: 9-20) days and 12 (range: 9-12) days in the BEAM and Mx-Mel arms, respectively; it was statistically significantly shorter in the BEAM arm (p = .001). CONCLUSION: This study demonstrates that the Mx-Mel regimen has similar efficacy and toxicity compared with the BEAM regimen. Although time to neutrophil engraftment was shorter in the BEAM arm, it did not result as significant transplant-related complications between the two regimens. The Mx-Mel regimen is seen as a good alternative with low toxicity and high efficacy.

Brentuximab vedotin and bendamustine: an effective salvage therapy for relapsed or refractory Hodgkin lymphoma patients.

The prognosis is poor for relapsed or refractory (R/R) classical Hodgkin Lymphoma (cHL) patients. The brentuximab vedotin (Bv) and bendamustine (B) combination has been used as a preferable salvage regimen in R/R cHL patient trials. We retrospectively evaluated response rates, toxicities, and the survival in R/R cHL patients treated with the BvB combination. In a multi-centre real-life study, 61 R/R HL patients received intravenous doses of 1.8 mg/kg Bv on the first day plus 90 mg/m2 B on the first and second days of a 21-day cycle as a second-line or beyond-salvage regimen. Patients' median age at BvB initiation was 33 (range: 18-76 years). BvB was given as median third-line treatment for a median of four cycles (range: 2-11). The overall and complete response rates were 82% and 68.9%, respectively. After BvB initiation, the median follow-up was 14 months, and one- and two-year overall survival rates were 85% and 72%, respectively. Grade 3/4 toxicities included neutropenia (24.6%), lymphopenia (40%), thrombocytopenia (13%), anaemia (13%), infusion reactions (8.2%), neuropathy (6.5%), and others. The BvB combination could be given as salvage regimen aiming a bridge to autologous stem cell transplant (ASCT), in patients relapse after ASCT or to transplant-ineligible patients with manageable toxicity profiles.

The effects of perindopril on QT duration and dispersion in patients with coronary slow flow.

Coronary slow flow (CSF) is characterized by delayed opacification of coronary arteries in the absence epicardial occlusive disease. QT duration and dispersion have been reported to be longer in patients with CSF. ACE inhibitors may improve CSF through positive effects on endothelial function. The study included 32 patients having CSF and 25 subjects having normal coronary arteries in coronary angiography. The patients were evaluated with 12-leads electrocardiography and echocardiography before and 3 months after treatment with perindopril. Compared to the control group, maximum corrected QT duration (QTcmax) (432.0 ± 28.9 vs. 407.0 ± 39.1 ms, p = 0.008) and QT dispersion (QTcD) (64.0 ± 16.5 vs. 37.3 ± 12.1 ms, p < 0.001), mitral inflow deceleration time (DT) (251.3 ± 49.4 vs. 218.8 ± 44.5 ms, p = 0.013), and isovolumetric relaxation time (IVRT) (115.8 ± 18.4 vs. 107.2 ± 22.9 ms, p < 0.001) were significantly longer and E/A ratio 0.85 ± 0.2 vs. 1.1 ± 0.3, p = 0.004) was lower in patients with CSF. QTcmax (to 407.0 ± 28.0 ms, p = 0.001), QTcD (to 44.5 ± 11.4 ms, p < 0.001), DT (to 221.6 ± 37.7 ms, p < 0.001) and IVRT (to 103.8 ± 16.1 ms, p < 0.001) were significantly decreased and E/A ratio (to 0.98 ± 0.3, p < 0.001) was significantly increased after treatment with perindopril. Coronary slow may be associated with prolonged QT interval and increased QT dispersion and impaired diastolic filling. Perindopril may be helpful in restoration of these findings.

The Spectrum of Beta-Thalassemia Mutations in Syrian Refugees and Turkish Citizens.

Introduction and objectives Neighboring the border between Turkey and Syria, Sanliurfa is one of the Turkish provinces with the highest number of Syrian refugees in our country. We aimed to find out the spectrum of beta-globin gene mutations in adult Turkish citizens and Syrian refugees with beta-thalassemia major. Results Of the participants, 35 patients (70%) were Turkish citizens and 15 patients (30%) were Syrian. The most common mutation in Turkish patients was found to be IVS-I-110 (G>A) with a frequency of 28.8%, followed by IVS-I-6 (T>C) with a frequency of 15.5%. Other common mutations were IVS-I-1 (G>A) and codon 39 (C>T) with frequencies of 11.1%. These four mutations accounted for 65.5% of all mutations in the Turkish cohort. The most common mutations in Syrian refugee patients were IVS-I-1 (G>A), IVS-II-1 (G>A), IVS-I-5 (G>C), and codon 5 (-CT), all with a frequency of 15.7%, accounting for 62.8% of all mutations in the Syrian patients. In the analysis, codon 5 (-CT) mutation (15.7% vs 0%, p=0.023) was found significantly higher in Syrian refugees compared to Turkish citizens. Discussion and conclusions A wide spectrum of mutations was detected in beta-thalassemia major patients living in the Sanliurfa region. Mutational profiles in Turkish and Syrian patients were found to be significantly different from each other. Because marriages between Syrian refugees and Turkish citizens are increasing in our region, the genetic findings and the mutational profiles in Turkish and Syrian patients obtained in this study are thought to become useful for future prenatal molecular diagnostic tests.

Evaluation of the predictive role of neutrophil/lymphocyte ratio in the diagnosis of lymphoma in patients with asymptomatic and isolated cervical lymphadenopathy.

INTRODUCTION: The diagnostic approach to patients with isolated asymptomatic cervical lymphadenopathy varies between excisional biopsy and follow-up. When the anamnesis, physical examination, laboratory and imaging findings are not sufficient to identify the etiology, an excisional biopsy is performed for the differential diagnosis between early-stage lymphoma and infectious or reactive causes. If the excisional biopsy, which may have some complications, is not performed, it may delay the diagnosis of lymphoma. This diagnostic challenge could be avoided by predictive markers. OBJECTIVES: This study was planned to determine the predictive value of neutrophil/lymphocyte ratio in the diagnosis of Hodgkin and non-Hodgkin limphoma in patients with asymptomatic, isolated cervical limphadenopathy and underwent excisional biopsy. METHODS: A total of 90 patients between the years 2016-2019 admitted to our clinics due to asymptomatic isolated cervical lymphadenopathy, present in at least 4 weeks with lympho nodes in pathological dimensions persisting in the cervical region, were included to our study. An excisional lympho node biopsy was performed in all 90 patients. RESULTS: Of the 90 patients who underwent excisional biopsy; 34 were diagnosed as reactive lymphadenopathy 30 were non-Hodgkin linphoma, and 26 were Hodgkin linphoma. A total of 56 (62.2%) patients were diagnosed as lymphoma, either Hodgkin or non-Hodgkin, while 34 patients (38.8%) were diagnosed as reactive lymphadenopathy. The median age, total whiteblood count, neutrophil count of the lymphoma groups were significantly higher than reactive lymphadenopathy group, whereas the lymphocyte count was significantly lower in the lymphoma patients. The median neutrophil/ lymphocyte ratio was 1.7 in the reactive lymphadenopathy group, 3.5 in the non-Hodgkin limphoma group, and 3.0 in the Hodgkin limphoma group (p< 0.001). CONCLUSION: According to the results of our study, neutrophil/lymphocyte ratio was significantly higher in patients who were admitted with isolated asymptomatic lymphadenopathy and were diagnosed with lymphoma, and who were diagnosed with early-stage Hodgkin and non- Hodgkin lymphoma compared to those who were found to have reactive lymphadenopathy. Neutrophil/lymphocyte ratio, which is a low-cost, fast and easy-to-access test, has a predictive value in the diagnosis of lymphoma in patients with asymptomatic lymphadenopathy.

The evaluation of patients with essential thrombocythemia in terms of risk of thrombosis.

OBJECTIVE: The aim of this study was to compare the incidence of factors associated with an increased risk of thrombosis in patients with essential thrombocythemia. METHODS: A total of 200 patients followed-up in our unit with a diagnosis of essential thrombocythemia in 13 years were analyzed retrospectively. RESULTS: Of the study participants, 60.5% were females and 39.5% were males, with an overall mean (±SD) age of 54.93 (±14.21) years. In 119 patients, Janus Kinase 2 was positive with 56.3% of cases. When two patient categories were defined as those with or without history of thrombosis, no significant differences were found in terms of Janus Kinase 2 positivity, mean age, as well as white blood cells and platelet counts (p>0.05). Also, no significant differences in thrombotic event incidence were found between patient categories defined on the basis of cut-off values for white blood cells (cut-off values of 15×103/mm3 and 8.7×103/mm3) and platelets (cut-off values of 1500×103/mm3) (p>0.05). CONCLUSION: Although our results are generally in line with the published data, some divergence from previous results has been observed with respect to risk factors for thrombotic events. Absence of a correlation between leukocytosis and thrombosis may be related with the significant decline in white blood cells after treatment. Also, a significant reduction in platelet counts occurring in association with treatment is linked with a lowered incidence of thrombosis. Janus Kinase 2-positive patients had a similar thrombosis frequency with that reported in the literature.

Conditioning Regimens for Relapsed/Refractory Lymphoma Patients Undergoing Autologous Stem Cell Transplantation: BEAM Versus High Dose ICE.

There are different drug combinations and conditioning regimens in lymphoma transplants. However, no randomized data is available to demonstrate the superiority of any regimen and the optimal choice is unknown. In this analysis, we compared the efficacy, toxicity and the survival outcomes of the BEAM and the high dose ICE (hdICE) conditioning regimens in relapsed NHL and relapsed/refractory Hodgkin Lymphoma patients undergoing auto-SCT. 83 patients with relapsed/refractory HL or relapsed NHL who were treated with Auto-SCT between 2006 and 2016, were analyzed retrospectively. 52 patients (62.7%) received BEAM, while 31 patients (37.3%) received hdICE. Between two groups there is no significant difference in age, gender, diagnosis, disease stage, chemosensitivity, ECOG performance status, time from diagnosis to transplant, salvage regimens and previous lines of chemotherapy. After a median of 59-month follow-up, PFS and OS rates of both groups were similar (5-year PFS was 51.6% in BEAM group, 48.8% in hdICE group, p = 0.71; 5-year OS was 58% in BEAM group, 54.8% in hdICE group, p = 0.93). The median neutrophil (11 vs. 10 days, p = 0.06) and platelet engraftment (13 vs. 11 days, p = 0.01) was faster and demand of transfusions were lesser in hdICE group (p = 0.03). However, severe renal toxicity was significantly higher in hdICE group in our study (p = 0.01). hdICE conditioning regimen may be used as an alternative to BEAM, with similar survival outcomes and toxicity profile, especially transplant centers that experience some difficulties in the availability of the carmustine.

A Real-life Turkish Experience of Venetoclax Treatment in High-risk Myelodysplastic Syndrome and Acute Myeloid Leukemia.

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

Efficacy and Safety of Ibrutinib Therapy in Patients with Chronic Lymphocytic Leukemia: Retrospective Analysis of Real-Life Data

Objective: This study aimed to retrospectively evaluate the efficacy, safety, and survival outcome of single-agent ibrutinib therapy in chronic lymphocytic leukemia patients. Materials and Methods: A total of 136 patients (mean age ± standard deviation: 64.6±10.3 years, 66.9% males) who had received at least one dose of ibrutinib were included in this retrospective multicenter, noninterventional hospital-registry study conducted at 33 centers across Turkey. Data on patient demographics, baseline characteristics, laboratory findings, and leukemia-cell cytogenetics were retrieved. Treatment response, survival outcome including overall survival (OS) and progression-free survival (PFS), and safety data were analyzed. Results: Overall, 36.7% of patients were categorized as Eastern Cooperative Oncology Group (ECOG) class 2-3, while 44.9% were in Rai stage 4. Fluorescence in situ hybridization revealed the presence of del(17p) in 39.8% of the patients. Patients received a median of 2.0 (range: 0-7) lines of pre-ibrutinib therapy. Median duration of therapy was 8.8 months (range: 0.4-58.0 months). The 1-year PFS and OS rates were 82.2% and 84.6%, respectively, while median PFS time was 30.0 (standard error, 95% confidence interval: 5.1, 20.0-40.0) months and median OS time was 37.9 (3.2, 31.5-44.2) months. Treatment response (complete or partial response), PFS time, and OS time were better with 0-2 lines versus 3-7 lines of prior therapy (p<0.001, p=0.001, and p<0.001, respectively), with ECOG class 0-1 versus class 2-3 (p=0.006, p=0.011, and p=0.001, respectively), and with Rai stage 0-2 versus 3-4 (p=0.002, p=0.001, and p=0.002, respectively). No significant difference was noted in treatment response rates or survival outcome with respect to the presence of comorbidity, bulky disease, or del(17p). While 176 adverse events (AEs) were reported in 74 (54.4%) patients, 46 of those 176 AEs were grade 3-4, including pneumonia (n=12), neutropenia (n=11), anemia (n=5), thrombocytopenia (n=5), and fever (n=5). Conclusion: This real-life analysis confirms the favorable efficacy and safety profile of long-term ibrutinib treatment while emphasizing the potential adverse impacts of poorer ECOG performance status, heavy treatment prior to ibrutinib, and advanced Rai stage on patient compliance, treatment response, and survival outcomes.

Noninvasive assessment of atherosclerosis in patients with isolated hypertension.

BACKGROUND: Endothelial dysfunction is considered the first stage in the development of atherosclerosis. Brachial artery flow-mediated dilatation (FMD) has been used to assess endothelial dysfunction. An impaired FMD response may reflect a vascular phenotype prone to atherosclerosis. The thickness of the common carotid intima-media (CIMT) as measured by ultrasound represents a marker of structural atherosclerosis. Recently, it has been shown that color M-mode propagation velocity measured along the origin of descending thoracic aorta (AVP) may reflect atherosclerosis. In this study, the effects of isolated hypertension on these atherosclerosis markers are investigated. METHODS: Fifty patients with newly diagnosed hypertension and forty healthy people were enrolled. Patients were evaluated with transthoracic echocardiography. Diastolic functions were evaluated by transmitral filling parameters of deceleration time (DT), E/A ratio, and isovolumetric relaxation time (IVRT). Carotid intima-media thickness, FMD, and AVP were measured. RESULTS: Age, gender, and BMI of both groups were similar. Compared to control group CIMT, DT and IVRT values were significantly higher, and FMD and AVP values were significantly lower in hypertensive patients. There were significant correlations between AVP and CIMT (r =-0.699, P < 0.001), AVP and FMD (r = 0.400, P < 0.001), and FMD and CIMT (r =-0.600, P < 0.001). Carotid intima-media thickness, AVP, and FMD were significantly correlated with systolic and diastolic blood pressures and DT and IVRT. CONCLUSIONS: In patients with isolated hypertension, AVP and FMD decrease and CIMT increases. In addition, CIMT is inversely correlated with AVP and FMD, and AVP is directly correlated with FMD.