Effectiveness of defibrotide in the prevention of hepatic venooclusive disease among adult patients receiving allogeneic hematopoietic cell transplantation: A retrospective single center experience

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is one of the most life-threatening early complications following hematopoietic cell transplantation (HCT). Due to the high mortality rate of severe VOD/SOS accompanied with multiorgan failure, there is a great interest in preventive strategies. The efficacy of defibrotide (DF) on the prevention of VOD/SOS has been clearly shown in high-risk pediatric patients, but evidence-based data on adults is scarce. In this report, we aimed to assess the impact of DF on the incidence of VOD/SOS in our center by posttransplant day 30 among patients who were treated with allogeneic HCT (allo-HCT). The study included a total of 56 patiens (28 males, 28 females). The median age of the study cohort was 43 (20-68). The daily dose of DF was 10 mg/kg and 25 mg/kg in 53 (94.6 %) and 3 (5.3 %) patients, respectively. Patients also recieved oral ursodeoxycolic acid (UDCA) 250 mg three-times daily started with conditioning until D + 90. Twenty-three (41.1 %) patients had at least one major EBMT-defined risk factor for development of VOD/SOS. One patient who belonged to a very high-risk group (with at least two major risk factors) developed very-severe VOD/SOS at posttransplant D + 20 and died as a result of multiorgan failure. The cumulative incidence of VOD/SOS at D + 30 was 1.9 %. Our findings indicate that 10 mg/kg daily intravenous DF combined with UDCA is quite effective in prevention of VOD/SOS in patients who underwent first allo-HSCT

Gilteritinib (XOSPATA (R)) in Turkey: Early access program results

Background And Objectives: Gilteritinib (XOSPATA (R), Astellas) is a type I oral FLT3 inhibitor, a tyrosine kinase AXL inhibitor, involved in both c-Kit and FMS-like tyrosine kinase 3 (FLT3) resistance. In the phase 3 ADMIRAL trial, gilteritinib was compared with the standard of care in (R/R) acute myeloid leukemia (AML) patients who harbored any FLT3 mutation and showed superior efficacy with regard to response and survival. Objectives: This research aimed to investigate the real-life efficacy and safety of gilteritinib in FLT3-positive R/R AML patients who were treated as a part of an early access program held in Turkey in April 2020 (NCT03409081). Results: The research included 17 R/R AML patients who had received gilteritinib from seven centers. The overall response rate was 100%. The most common adverse events were anemia and hypokalemia (7 patients, 41.2%). Grade 4 thrombocytopenia was observed in one patient only (5.9%), leading to permanent treatment discontinuation. Patients with peripheral edema had a 10.47 (95% CI: 1.64-66.82) times higher risk of death than those without peripheral edema (p<0.05). Conclusion: This research showed that patients with febrile neutropenia and peripheral edema were at a high risk of death when compared to patients without febrile neutropenia and peripheral edema

“The Lolelaplap (Marshall Islands) in Us: Sailing West to East (Ralik→Ratak) to These Our Atolls (Aelon Kein Ad) Ad Jolet Jen Anij (Our Blessed Inheritance from God)”

This paper discusses the expansion of Oceania through a Marshallese indigenous lens as a focal point. It explains that decolonizing methodologies allows reclaiming of space for mental liberation and reassurement of constitutional rights. It highlights similar occurrences of decolonization practices meeting resistance in the 21st century all while strengthening the human right argument that no human deserves any less than their fellow human brothers and sisters. It argues that an indigenous imagery can only be viewed through an indigenous lens where the researches’ level of purity is retained and unfiltered. It nevertheless argues that Marshallese ethnolinguistics reveal the same cultural practices in America, Judeo-Christianity, and Oceania thus dictating the reality that “we are the same not withstanding one stays here and one there (Bedbedjin Bedbedjen, Bedbedjinma wot Kwe)”. It further explains the importance in these similarities and how Marshallese spirituality predates introduced American Judeo-Christianity despite the latter attempting to marginalize the former. It concludes by stating that Marshallese contributions on the global stage are rooted in that culture of love (IaKwe) which is echoed by the custom(s) revealing the significance of Marshallese validation academically, spiritually, economically, & socially to prevent institutionalized discrimination. This paper ends stating that the agency to know one’s self and how one should fit in the world, is a human right in itself and Marshallese are entitled to this sense of self worth through knowing thy self by thy self where real thinking takes place in one’s own mind as we all live our own lives

Defining the role of sticky platelet syndrome in patients with acute coronary syndrome

Tıpta Uzmanlık TeziTromboembolizm gelişmiş ülkelerde önde gelen morbidite ve mortalite nedenidir. Tanımlanmış hiperkoagülabilitesi olan hastaların yarıdan fazlasında doğumsal veya edinsel koagülasyon veya trombosit bozukluğu bulunur. Yapışkan trombosit sendromu adenozin difosfat ve/veya epinefrine karşı in vivo trombosit hiperreaktivitesi ile karakterize doğumsal bir trombosit bozukluğudur. Yapışkan trombosit sendromu birçok arteryel ve venöz trombozdan sorumlu olan, sık rastlanan, tanı ve tedavisi kolay bir trombosit bozukluğudur. Çalışmamızın ilk basamağında yapışkan trombosit sendromu'nun tanımlanmasında kullanılan farklı konsantrasyonlarda adenozin difosfat ve epinefrin uyarısı sonrası kendi laboratuvarımızın normal agregasyon ve sekresyon değerlerini saptadık. Daha sonra akut koroner sendrom öyküsü olan ve anjiografik olarak koroner arterleri hastalarda yapışkan trombosit sendromu'nun rolünü bulmaya çalıştık. Trombositlerin agregasyon ve sekresyon reaksiyonları lümiagregometri yöntemi kullanılarak eş zamanlı kaydedildi. Çalışmamıza 49 sağlıklı kontrol ve akut koroner sendrom tanılı 23 hasta dahil edildi. 7 (%30) hastada yapışkan trombosit sendromu saptandı. Biri hariç tüm agonist konsantrasyonlarında agregasyon ve sekresyon yanıtları arasında anlamlı korelasyon izledik. Bulgularımız agregasyon ve sekresyon bağlamında trombosit hiperreaktivitesinin global bir fenomen olduğunu ve seçilmiş hasta grubumuzda yapışkan trombosit sendromu'nun sık rastlanan bir bozukluk olduğunu gösterdi.AbstractThromboembolism is the leading cause of morbidity and mortality in developed countries. More than half of patients with defined hypercoagulability have a hereditary or aquired coagulation or platelet disorder that caused thrombosis. The sticky platelet syndrome is a hereditary platelet defect characterized with increased in vivo platelet hyperreactivity to adenosine diphosphate and/or epinephrine . Sticky platelet syndrome is a common, easy to diagnose and easy to treat platelet defect that is responsible for many arteriel and venous thrombotic events. At first step of our study we found normal aggregation and secretion ranges of our own laboratory after stimulation with different concentrations of adenosine diphosphate and epinephrine which was used to define sticky platelet syndrome . Thereafter we tried to find out the role of sticky platelet syndrome among patients with a history of acute coronary syndrome and angiographically normal coronary arteries. Aggregation and secretion reactions of platelets were recorded at the same time using lumiaggregometric method. Our study included 49 healty controls and 23 patients with acute coronary syndrome. 7 (30%) patients were found to have sticky platelet syndrome. We observed significant correlation between aggregation and secretion responses on all but one agonist concentrations. Our findings indicate that platelet hyperreactivity could be a global phenomenon in terms of aggregation and secretion, and sticky platelet syndrome is a common defect in our selected patient population

Effectiveness of defibrotide in the prevention of hepatic venooclusive disease among adult patients receiving allogeneic hematopoietic cell transplantation: A retrospective single center experience

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is one of the most life-threatening early complications following hematopoietic cell transplantation (HCT). Due to the high mortality rate of severe VOD/SOS accompanied with multiorgan failure, there is a great interest in preventive strategies. The efficacy of defibrotide (DF) on the prevention of VOD/SOS has been clearly shown in high-risk pediatric patients, but evidence-based data on adults is scarce. In this report, we aimed to assess the impact of DF on the incidence of VOD/SOS in our center by posttransplant day 30 among patients who were treated with allogeneic HCT (allo?HCT). The study included a total of 56 patiens (28 males, 28 females). The median age of the study cohort was 43 (20?68). The daily dose of DF was 10 mg/kg and 25 mg/kg in 53 (94.6 %) and 3 (5.3 %) patients, respectively. Patients also recieved oral ursodeoxycolic acid (UDCA) 250 mg three-times daily started with conditioning until D + 90. Twenty-three (41.1 %) patients had at least one major EBMT-defined risk factor for development of VOD/SOS. One patient who belonged to a very high-risk group (with at least two major risk factors) developed very-severe VOD/SOS at posttransplant D + 20 and died as a result of multiorgan failure. The cumulative incidence of VOD/SOS at D + 30 was 1.9 %. Our findings indicate that 10 mg/kg daily intravenous DF combined with UDCA is quite effective in prevention of VOD/SOS in patients who underwent first allo-HSCT. © 202

Differences in platelet-leukocyte aggregates among subtypes of acute cerebral ischemia

Background: Acute cerebral ischemia is caused by different pathophysiological mechanisms. The role of platelets and other blood cells can be different among the stroke subtypes. Methods: Seventy-two patients with acute ischemic cerebrovascular disease, including 31 patients with large vessel disease, 21 patients with cardioembolic disease, and 20 patients with small vessel disease, were evaluated. P-selectin (CD62P) expression and platelet leukocyte aggregates were measured with flow cytometry at the acute phase after the ischemic event. Markers were also measured in 37 control subjects. In all subjects, the serum high-sensitivity C-reactive protein (CRP) was also measured. Results: The platelet-monocyte aggregates (PMA) and platelet-granulocyte aggregates (PGA) in the large vessel disease group were higher than in control group (P=0.002, and P < 0.0001, respectively). The PMA and PGA in the small vessel disease group were also higher than in the control group (P=0.004 and P < 0.0001, respectively). In contrast, in the cardioembolic disease group, the PMA and PGA were not significantly different from the control group. CD62P expression was higher in all of the patient groups relative to the control group (P < 0.05 for all comparisons). Serum CRP levels were also higher in all of the patient groups than in the control group (P < 0.0001 for all comparisons). Conclusions: In contrast to large vessel and small vessel disease, it seems that platelet-leukocyte association does not play a crucial role in the pathogenesis of cardioembolic stroke. (C) 2011 Elsevier B.V. All rights reserved.Trakya UniversityTrakya UniversityThis study was supported by grants from The Research Fund of Trakya University. We wish to thank S. Azcan for her valuable technical assistance

Prospective registry of adult patients receiving therapeutic plasma exchange with a presumptive diagnosis of thrombotic microangiopathy (TMA): the Turkish hematology research and education group (ThREG)-TMA02 study

Thrombotic microanjiopathy (TMA) is a pathological diagnosis characterized by abnormalities of small vessels leading to microvascular thrombosis of arterioles and capillaries. The current prospective, non-interventional, multicenter study aimed to define the distribution of different TMA forms in adult Turkish patients who were referred for therapeutic plasma exchange (TPE) for presumptive diagnosis of TMA. Patients with serum ADAMTS13 activity 10 %, normal renal function and no secondary TMA were treated as unclassified TMA. The study included a total of 80 patients (women: 50; man: 30) with a median age of 48 (20?74). Detailed evaluation at 1 month after hospital admission revealed aTTP, secondary TMA, infection/complement-associated hemolytic uremic syndrome and unclassified TMA in 29 (36.2 %), 22 (27.5 %), 23 (28.8 %) and 6 (7.5 %) patients respectively. As subclassification of various TMAs will dictate specific therapy, proper diagnosis in a timely manner is of utmost clinical significance. © 2022Alexion Pharmaceuticals: 100064The study was sponsored by Alexion Pharmaceuticals as an investigator initiated trial (Tracking Number: 100064)