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Total 198 results found since Jan 2013.
Comparison between Automated Erythrocytopharesis (AECP) and Manual Exchange Transfusion (M-Ex)) in Reducing Hb-S and in Recovery of Acute Chest Syndrome and Other Acute Presentations of Sickle Cell Disease Patients
Conclusions:Erythrocytopharesis (Automated RBC exchange) is effective, quick and safe procedure that is life saving for many patients with ACS and is associated with less difficulties and complications if compared with the manual exchange. Because SCA is a national problem in Saudi Arabia and acute chest syndrome and other acute major complications comprehensively kill SCD patients, Automated Erythocytopharesis should be available nation-wide like dialysis machines at all large hospitals in all cities and should be distributed according to the prevalence of SCA in the area or location.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Ahmed, S. Y., Saleh, S. M., Hameed, M. S., Ragheb, A. M., Abbas, T. M., Fadel, A., Hassan, S., Baden, H. S., Tayeb, K. I., Bakshi, N., El Yamany, G., Alotaibi, S. B. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical Source Type: research
Description of the First Cases with ADAMTS13 Mutations in Hungary
CONCLUSIONS:Clinical and molecular characterization of the first 5 Hungarian cases with ADAMTS13 mutations was presented. Compound heterozygous, damaging ADAMTS13 mutations with deficient plasma ADAMTS13 activity were associated with serious clinical consequences. In case of thrombotic microangiopathy in young patients with stroke, or HELLP syndrome, complete workup for ADAMTS13 activity and inhibitors is necessary, and genetic analysis should be considered. Three new ADAMTS13 mutations (c.1016_1017delCA, c.3199T>A , c.2839C>T) were identified and shown to be associated with decreased plasma ADAMTS13 activity.Disclos...
Source: Blood - November 21, 2018 Category: Hematology Authors: Reti, M., Sinkovits, G., Cseprekal, O., Csuka, D., Szilagyi, A., Farkas, Z., Klucsik, Z., Szederjesi, A., Wagner, L., Reusz, G., Kremer Hovinga, J. A., Rigo, J., Masszi, T., Prohaszka, Z. Tags: 311. Disorders of Platelet Number or Function Source Type: research
Thrombotic Thrombocytopenic Purpura in Oman; Manifestations and Outcome. Retrospective Study
Conclusion:This is the first study that demonstrates the demographic, clinical and survival rate of TTP patients in Oman, and provides a general picture of the TTP patients in our countryDisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Al Hashmi, K. N., Al Dowaiki, S. Tags: 311. Disorders of Platelet Number or Function Source Type: research
Umbilical Cord Blood Mononuclear Cells for Ex-Vivo Gene Therapy
This study was supported by the grant of Russian Science Foundation No 16-15-00010. Kazan Federal University was supported by the Russian Government Program of Competitive Growth.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Bashirov, F. V., Salafutdinov, I. I., Sokolov, M. E., Izmailov, A. A., Markosyan, V. A., Fadeev, F. O., Rizvanov, A., Islamov, R. I. Tags: 801. Gene Therapy and Transfer Source Type: research
The Effect of Health Care Disparities on Complications and Mortality in Sickle Cell Disease
Discussion:The data indicates that the rate of complications from SCD have risen since 1999. With newer therapies and better understanding, the life expectancy of SCD patients has risen over time, nearly doubling from 1951 to 2018. The increased frequency of complications may be attributed to better survivorship and a rising number of older SCDs patients. However, our data also suggests that insurance status plays a significant role in the complication rate of SCD. The uninsured and patients with Medicaid have significantly increased risk of developing disease complications and resultant mortality. This could be the result...
Source: Blood - November 21, 2018 Category: Hematology Authors: Perimbeti, S. P., Hou, K. Y., Ramanathan, S., Woodard, A., Kyung, D., Wang, Q., Crilley, P. A., Ward, K., Styler, M. Tags: 903. Outcomes Research-Non-Malignant Hematology Source Type: research
Characterization and treatment of congenital thrombotic thrombocytopenic purpura
In conclusion, prespacer mutations are associated with earlier development of cTTP symptoms. Prophylactic ADAMTS13 replacement decreases the risk of end-organ damage such as ischemic stroke and resolved previously unrecognized symptoms in patients with nonovert disease.
Source: Blood - April 10, 2019 Category: Hematology Authors: Alwan, F., Vendramin, C., Liesner, R., Clark, A., Lester, W., Dutt, T., Thomas, W., Gooding, R., Biss, T., Watson, H. G., Cooper, N., Rayment, R., Cranfield, T., van Veen, J. J., Hill, Q. A., Davis, S., Motwani, J., Bhatnagar, N., Priddee, N., David, M., Tags: Thrombocytopenia, Platelets and Thrombopoiesis, Clinical Trials and Observations Source Type: research
Hydroxyurea reduces cerebral metabolic stress in patients with sickle cell anemia
Chronic transfusion therapy (CTT) prevents stroke in selected patients with sickle cell anemia (SCA). We have shown that CTT mitigates signatures of cerebral metabolic stress, reflected by elevated oxygen extraction fraction (OEF), which likely drives stroke risk reduction. The region of highest OEF falls within the border zone, where cerebral blood flow (CBF) nadirs; OEF in this region was reduced after CTT. The neuroprotective efficacy of hydroxyurea (HU) remains unclear. To test our hypothesis that patients receiving HU therapy have lower cerebral metabolic stress compared with patients not receiving disease-modifying t...
Source: Blood - May 29, 2019 Category: Hematology Authors: Fields, M. E., Guilliams, K. P., Ragan, D., Binkley, M. M., Mirro, A., Fellah, S., Hulbert, M. L., Blinder, M., Eldeniz, C., Vo, K., Shimony, J. S., Chen, Y., McKinstry, R. C., An, H., Lee, J.-M., Ford, A. L. Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis Source Type: research
EGFL7 ligates {alpha}v{beta}3 integrin to enhance vessel formation
Angiogenesis, defined as blood vessel formation from a preexisting vasculature, is governed by multiple signal cascades including integrin receptors, in particular integrin αVβ3. Here we identify the endothelial cell (EC)-secreted factor epidermal growth factor-like protein 7 (EGFL7) as a novel specific ligand of integrin αVβ3, thus providing mechanistic insight into its proangiogenic actions in vitro and in vivo. Specifically, EGFL7 attaches to the extracellular matrix and by its interaction with integrin αVβ3 increases the motility of EC, which allows EC to move on a sticky underground du...
Source: Blood - April 11, 2013 Category: Hematology Authors: Nikolic, I., Stankovic, N. D., Bicker, F., Meister, J., Braun, H., Awwad, K., Baumgart, J., Simon, K., Thal, S. C., Patra, C., Harter, P. N., Plate, K. H., Engel, F. B., Dimmeler, S., Eble, J. A., Mittelbronn, M., Schafer, M. K., Jungblut, B., Chavakis, E Tags: Vascular Biology Source Type: research
A specific antidote for dabigatran: functional and structural characterization
Dabigatran etexilate is a direct thrombin inhibitor and used widely as an anticoagulant for the prevention of stroke in patients with atrial fibrillation. However, anticoagulation therapy can be associated with an increased risk of bleeding. Here, we present data on the identification, humanization, and in vitro pharmacology of an antidote for dabigatran (aDabi-Fab). The X-ray crystal structure of dabigatran in complex with the antidote reveals many structural similarities of dabigatran recognition compared with thrombin. By a tighter network of interactions, the antidote achieves an affinity for dabigatran that is ~350 ti...
Source: Blood - May 2, 2013 Category: Hematology Authors: Schiele, F., van Ryn, J., Canada, K., Newsome, C., Sepulveda, E., Park, J., Nar, H., Litzenburger, T. Tags: Plenary Papers, Thrombosis and Hemostasis Source Type: research
Only severe thrombocytopenia results in bleeding and defective thrombus formation in mice
Platelets are essential mediators of hemostasis and thrombosis. Platelet counts (PCs) in humans average 250 platelets/nL, but it is not entirely clear how platelet numbers affect hemostasis and occurrence of thrombotic events. Mice, displaying PCs of ~1000 platelets/nL, are widely used to assess platelet function in (patho-)physiology, but also in this species, the significance of PC for hemostasis and thrombotic disease is not established. We reduced PCs in mice to defined ranges between 0 and 1000 platelets/nL by platelet-depleting antibodies and challenged them in different arterial thrombosis models: the transient midd...
Source: Blood - June 13, 2013 Category: Hematology Authors: Morowski, M., Vogtle, T., Kraft, P., Kleinschnitz, C., Stoll, G., Nieswandt, B. Tags: Thrombocytopenia, Platelets and Thrombopoiesis, Thrombosis and Hemostasis Source Type: research
Increased risk of CVD after VT is determined by common etiologic factors
In conclusion, individuals with VT had an increased risk of CVD. This could be explained by common etiologic factors.
Source: Blood - June 13, 2013 Category: Hematology Authors: Roach, R. E. J., Lijfering, W. M., Flinterman, L. E., Rosendaal, F. R., Cannegieter, S. C. Tags: Free Research Articles, Thrombosis and Hemostasis Source Type: research
(Dis)solving the stroke problem by vWF inhibition?
Source: Blood - June 20, 2013 Category: Hematology Authors: Nieswandt, B., Stoll, G. Tags: Free Research Articles INSIDE BLOOD Source Type: research
Reperfusion of cerebral artery thrombosis by the GPIb-VWF blockade with the Nanobody ALX-0081 reduces brain infarct size in guinea pigs
Thrombolytic therapy is the cornerstone of treatment of acute atherothrombotic ischemic stroke but is associated with brain hemorrhage; antiplatelet therapy has limited efficacy and is still associated with intracranial bleeding. Therefore, new antithrombotic approaches with a better efficacy/safety ratio are required. We have assessed the effect of ALX-0081, a Nanobody against the A1 domain of von Willebrand factor (VWF) that blocks VWF binding to GPIb, of the thrombolytic agent recombinant tissue plasminogen activator (rtPA), and of the GPIIb/IIIa antagonist tirofiban, in a middle cerebral artery (MCA) thrombosis model i...
Source: Blood - June 20, 2013 Category: Hematology Authors: Momi, S., Tantucci, M., Van Roy, M., Ulrichts, H., Ricci, G., Gresele, P. Tags: Platelets and Thrombopoiesis, Thrombosis and Hemostasis Source Type: research
Comparison of vascular growth factors in the murine brain reveals placenta growth factor as prime candidate for CNS revascularization
Vascular bypass procedures in the central nervous system (CNS) remain technically challenging, hindered by complications and often failing to prevent adverse outcome such as stroke. Thus, there is an unmet clinical need for a safe and effective CNS revascularization. Vascular endothelial growth factors (VEGFs) are promising candidates for revascularization; however, their effects appear to be tissue-specific and their potential in the CNS has not been fully explored. To test growth factors for angiogenesis in the CNS, we characterized the effects of endothelium-specific growth factors on the brain vasculature and parenchym...
Source: Blood - August 1, 2013 Category: Hematology Authors: Gaal, E. I., Tammela, T., Anisimov, A., Marbacher, S., Honkanen, P., Zarkada, G., Leppanen, V.-M., Tatlisumak, T., Hernesniemi, J., Niemela, M., Alitalo, K. Tags: Vascular Biology, Gene Therapy Source Type: research
Antithrombotic actions of statins involve PECAM-1 signaling
Statins are widely prescribed cholesterol-lowering drugs that are a first-line treatment of coronary artery disease and atherosclerosis, reducing the incidence of thrombotic events such as myocardial infarction and stroke. Statins have been shown to reduce platelet activation, although the mechanism(s) through which this occurs is unclear. Because several of the characteristic effects of statins on platelets are shared with those elicited by the inhibitory platelet adhesion receptor PECAM-1 (platelet endothelial cell adhesion molecule-1), we investigated a potential connection between the influence of statins on platelet f...
Source: Blood - October 31, 2013 Category: Hematology Authors: Moraes, L. A., Vaiyapuri, S., Sasikumar, P., Ali, M. S., Kriek, N., Sage, T., Gibbins, J. M. Tags: Platelets and Thrombopoiesis Source Type: research
