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Total 198 results found since Jan 2013.
Degree of anemia, indirect markers of hemolysis, and vascular complications of sickle cell disease in Africa
In conclusion, severe anemia at steady state in SCD patients living in West and Central Africa is associated with elevated TRV, microalbuminuria, and leg ulcers, but these vascular complications are not independently associated with indirect markers of increased hemolysis. Other mechanisms leading to anemia, including malnutrition and infectious diseases, may also play a role in the development of SCD vasculopathy.
Source: Blood - November 16, 2017 Category: Hematology Authors: Dubert, M., Elion, J., Tolo, A., Diallo, D. A., Diop, S., Diagne, I., Sanogo, I., Belinga, S., Guifo, O., Wamba, G., Ngo Sack, F., Boidy, K., Kamara, I., Traore, Y., Diakite, C. O., Gbonon, V., Faye, B. F., Seck, M., Deme Ly, I., Chelo, D., NGuetta, R., D Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis Source Type: research
CK2{beta} regulates thrombopoiesis and Ca2+-triggered platelet activation in arterial thrombosis
Platelets, anucleated megakaryocyte (MK)-derived cells, play a major role in hemostasis and arterial thrombosis. Although protein kinase casein kinase 2 (CK2) is readily detected in MKs and platelets, the impact of CK2-dependent signaling on MK/platelet (patho-)physiology has remained elusive. The present study explored the impact of the CK2 regulatory β-subunit on platelet biogenesis and activation. MK/platelet-specific genetic deletion of CK2β (ck2β–/–) in mice resulted in a significant macrothrombocytopenia and an increased extramedullar megakaryopoiesis with an enhanced proportion of prematur...
Source: Blood - December 21, 2017 Category: Hematology Authors: Münzer, P., Walker-Allgaier, B., Geue, S., Langhauser, F., Geuss, E., Stegner, D., Aurbach, K., Semeniak, D., Chatterjee, M., Gonzalez Menendez, I., Märklin, M., Quintanilla-Martinez, L., Salih, H. R., Litchfield, D. W., Buchou, T., Kleins Tags: Platelets and Thrombopoiesis, Thrombosis and Hemostasis Source Type: research
Unwinding the path from anemia to stroke
Source: Blood - March 1, 2018 Category: Hematology Authors: Wood, J. C. Tags: Free Research Articles COMMENTS Source Type: research
Red cell exchange transfusions lower cerebral blood flow and oxygen extraction fraction in pediatric sickle cell anemia
Blood transfusions are the mainstay of stroke prevention in pediatric sickle cell anemia (SCA), but the physiology conferring this benefit is unclear. Cerebral blood flow (CBF) and oxygen extraction fraction (OEF) are elevated in SCA, likely compensating for reduced arterial oxygen content (CaO2). We hypothesized that exchange transfusions would decrease CBF and OEF by increasing CaO2, thereby relieving cerebral oxygen metabolic stress. Twenty-one children with SCA receiving chronic transfusion therapy (CTT) underwent magnetic resonance imaging before and after exchange transfusions. Arterial spin labeling and asymmetric s...
Source: Blood - March 1, 2018 Category: Hematology Authors: Guilliams, K. P., Fields, M. E., Ragan, D. K., Eldeniz, C., Binkley, M. M., Chen, Y., Comiskey, L. S., Doctor, A., Hulbert, M. L., Shimony, J. S., Vo, K. D., McKinstry, R. C., An, H., Lee, J.-M., Ford, A. L. Tags: Pediatric Hematology, Sickle Cell Disease, Transfusion Medicine, Free Research Articles, Red Cells, Iron, and Erythropoiesis Source Type: research
Inflammation, von Willebrand factor, and ADAMTS13
Increasing evidence indicates that inflammation can cause thrombosis by a von Willebrand factor (VWF)-mediated mechanism that includes endothelial activation, secretion of VWF, assembly of hyperadhesive VWF strings and fibers, cleavage by ADAMTS13, and adhesion and deposition of VWF-platelet thrombi in the vasculature. This mechanism appears to contribute to thrombosis not only in small vessels, but also in large vessels. Inflammation and VWF contribute to atherogenesis and may contribute to arterial and venous thrombosis as well as stroke. Elucidation of the mechanism will hopefully identify new targets and suggest new ap...
Source: Blood - July 12, 2018 Category: Hematology Authors: Chen, J., Chung, D. W. Tags: Platelets and Thrombopoiesis, Thrombosis and Hemostasis, Vascular Biology, Review Articles, Review Series Source Type: research
Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome
Rivaroxaban is an effective and safe alternative to warfarin in patients with atrial fibrillation and venous thromboembolism. We tested the efficacy and safety of rivaroxaban compared with warfarin in high-risk patients with thrombotic antiphospholipid syndrome. This is a randomized open-label multicenter noninferiority study with blinded end point adjudication. Rivaroxaban, 20 mg once daily (15 mg once daily based on kidney function) was compared with warfarin (international normalized ratio target 2.5) for the prevention of thromboembolic events, major bleeding, and vascular death in patients with antiphospholipid syndro...
Source: Blood - September 27, 2018 Category: Hematology Authors: Pengo, V., Denas, G., Zoppellaro, G., Jose, S. P., Hoxha, A., Ruffatti, A., Andreoli, L., Tincani, A., Cenci, C., Prisco, D., Fierro, T., Gresele, P., Cafolla, A., De Micheli, V., Ghirarduzzi, A., Tosetto, A., Falanga, A., Martinelli, I., Testa, S., Barce Tags: Plenary Papers, Thrombosis and Hemostasis, Clinical Trials and Observations Source Type: research
Testing and monitoring direct oral anticoagulants
Direct oral anticoagulants (DOACs) have significantly improved the care of patients requiring anticoagulation. With similar or better efficacy and safety outcomes and easier use in the outpatient setting compared with the standard-of-care vitamin K antagonists and low molecular weight heparin, DOACs are now endorsed as first-line treatment of indications including prevention of stroke and systemic embolism in nonvalvular atrial fibrillation and treatment of venous thromboembolism. DOACs are easy-to-use oral agents that offer simple dosing and short half-lives, with no need to test levels because of the wide therapeutic win...
Source: Blood - November 8, 2018 Category: Hematology Authors: Connors, J. M. Tags: Perspectives, Thrombosis and Hemostasis Source Type: research
Rivaroxaban Thromboprophylaxis in High-Risk Ambulatory Cancer Patients Receiving Systemic Therapy: Results of a Randomized Clinical Trial (CASSINI)
Conclusions: Rivaroxaban significantly reduced VTE and VTE-related death during the on-treatment period but not during the full study period; over one-third of events occurred post discontinuation of study drug. The incidence of major bleeding was low. The Khorana risk score cut-off of ≥2 identified cancer patients at high risk of thrombotic events both at baseline (4.53%) and during study (8.79% with additional 1.66% arterial events in placebo group). These results should inform future recommendations regarding thromboprophylaxis in at-risk ambulatory cancer patients.(Funded by Janssen; ClinicalTrials.gov number, NCT02...
Source: Blood - November 21, 2018 Category: Hematology Authors: Khorana, A. A., Soff, G. A., Kakkar, A. K., Vadhan-Raj, S., Riess, H., Wun, T., Streiff, M. B., Garcia, D. A., Liebman, H. A., Belani, C., O'Reilly, E. M., Patel, J. N., Yimer, H. A., Wildgoose, P., Burton, P., Vijapurkar, U., Kaul, S., Eikelboom, J., McB Tags: Late-Breaking Abstracts Session Source Type: research
Clinical, Laboratory, and Genetic Risk Factors for Thrombosis in Sickle Cell Disease
In conclusion, thrombotic events are common, occurring in 18% of SCD patients over a 10-year follow-up period. HbSS/Sβ0-thalassemia, frequent hospitalizations, kidney disease, and higher systolic blood pressures and AST concentrations were risk factors for thrombosis. Genome-wide marker array analysis points to a potential role of thrombomodulin in SCD-related thrombotic events based on replicated risk variants in THBD. Future studies integrating clinical, laboratory and genetic risk factors may improve our understanding for thrombosis and guide intervention practices in SCD.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Srisuwananukorn, A., Raslan, R., Zhang, X., Shah, B. N., Han, J., Gowhari, M., Jain, S., Molokie, R. E., Gordeuk, V. R., Saraf, S. L. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Organ Damage and Clinical Complications in Sickle Cell Disease Source Type: research
Reduced Cerebral Metabolic Rate of Oxygen in Adults with Sickle Cell Disease
Conclusion:We observed reduced CMRO2 in patients with SCD compared to healthy controls due to low OEF. A reduced CMRO2 could pose a risk for ischemia, despite high flow rate delivering oxygen, because of low OEF. This is supported by the fact that the silent cerebral infarcts are located in regions with the lowest CMRO2. We postulate that patients with SCD have a reduced capacity to increase the OEF in regions with inadequate CBF resulting in local ischemia and local infarction. The pathogenesis of the reduced OEF remains unclear but could be related to arteriovenous shunting whereby there is insufficient time for oxygen t...
Source: Blood - November 21, 2018 Category: Hematology Authors: Vaclavu, L., Petersen, E. T., VanBavel, E. T., Majoie, C. B., Nederveen, A. J., Biemond, B. J. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Organ Damage and Clinical Complications in Sickle Cell Disease Source Type: research
Determination of the Structural Changes Accompanying Binding of Protein S to Factor IXa
Conclusion: Based on our preliminary data, we conclude that PS binds strongly with FIXa, and the complex is stabilized by a shift in α-helical content as shown in Figure 1.Figure.1 shows the CD spectral change of PS (curve 1, right panel) due to addition of different concentration of FIXa (curve 2-8, right panel).DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Chatterjee, S., Guidry, J. J., Plautz, W. E., Watt, T., Majumder, R. Tags: 321. Blood Coagulation and Fibrinolytic Factors: Structural Biology of Coagulation Proteins Source Type: research
High Molecular Weight Kininogen Contributes to End-Organ Damage and Mortality in a Mouse Model of Sickle Cell Disease
Conclusions: These data indicate that HK deficiency attenuates chronic inflammation, kidney failure, and heart hypertrophy, and improves survival of sickle cell mice.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Sparkenbaugh, E., Wilson, K., Kasztan, M., Pollock, D. M., McCrae, K. R., Pawlinski, R. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Sickle Cell Disease-Role of Coagulation and Inflammation in Pathophysiology Source Type: research
Burden of Hospital Readmissions for Venous Thromboembolism Among Patients with Cancer
Conclusions: In this real-world study, many patients hospitalized for cancer experienced a VTE event requiring re-hospitalization, which was the highest proportion of readmitted patients among acute medical illnesses analyzed in this study, with almost 30% readmitted within 30 days of post-discharge. Total costs of readmissions were substantial, as high as $41K for resubmissions due to primary diagnosis of DVT/PE. Improvement in VTE prophylaxis for patients with cancer may reduce the risk and frequency of VTE, and thus hospital readmissions, reducing the clinical and economic burden of VTE in this patient population.Sponso...
Source: Blood - November 21, 2018 Category: Hematology Authors: Amin, A. J., Deitelzweig, S., Lin, J., Lingohr-Smith, M., Menges, B., Neuman, W. R. Tags: 901. Health Services Research-Non-Malignant Conditions: Thrombosis and Anticoagulation Source Type: research
Genotype-Phenotype Correlation in Congenital TTP: New Insights from a Multicentre Study with 121 Patients
Conclusion: At present, the Hereditary TTP Registry is the largest cohort on cTTP, to our knowledge. The large number of different mutations as well as confounding factors, including multi-ethnic and geographical factors in our internationally compiled patient cohort make unravelling the genotype-phenotype correlation in cTTP challenging. Yet, for c.4143_4144dupA, we can conclude that homozygous patients with ADAMTS13 c.4143_4144dupA are clinically less severely affected than compound heterozygotes due to the later onset of the disease in homozygotes, whereas the clinical characteristics are similar between compound hetero...
Source: Blood - November 21, 2018 Category: Hematology Authors: Van Dorland, A. A., Mansouri Taleghani, M., Friedman, K. D., George, J., Hrachovinova, I., Knobl, P., Lammle, B., Matsumoto, M., von Krogh, A. S., Schneppenheim, R., Aebi-Huber, I., Lukas, B., Cermakova, Z., Quist-Paulsen, P., Terrell, D., Vesely, S. K., Tags: 311. Disorders of Platelet Number or Function: Advances in the Treatment of TTP and HIT Source Type: research
Outcome of 472 Chronic Myeloid Leukemia Patients Treated with Frontline Nilotinib: A Gimema CML WP Analysis
CONCLUSION: Nilotinib as first-line treatment of newly diagnosed CP CML pts showed high rates of deep molecular responses, few progressions to AP/BP, and a high OS. Deep molecular response were similar in all age groups; as expected, ATEs were more frequent in pts > 60 y. These data suggest that: in pts > 60 y, the high efficacy of nilotinib should be weighed against its potential toxicity; in pts < 60 years, nilotinib may be a very good choice, with high efficacy and low toxicity.DisclosuresGugliotta: Novartis: Honoraria; Pfizer: Honoraria; Incyte: Honoraria; Bristol-Myers Squibb: Honoraria. Castagnetti: Bristol ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Gugliotta, G., Castagnetti, F., Breccia, M., Iurlo, A., D'Adda, M., Stagno, F., Levato, L., Martino, B., Lunghi, F., Tiribelli, M., Rege Cambrin, G., Abruzzese, E., Rupoli, S., Gozzini, A., Pregno, P., Salvucci, M., Trabacchi, E., Caocci, G., Scortechini, Tags: 632. Chronic Myeloid Leukemia: Therapy: First Line Trials and Prognostic Factors of Treatment-Free Remission Source Type: research
