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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

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

Oxidative Modified Fibrinogen in Cardiovascular Diseases
ConclusionsThe results show that the degree of impairment of fibrinogen functions in the cardiovascular diseases is related to the level of oxidative stress. Characterization of oxidative fibrinogen modification and its precise meaning to the function of fibrinogen in hemostasis appears to be extremely helpful to better understanding of thrombotic/bleeding complications linked with various cardiovascular diseases.AcknowledgementsThis work was supported by the Ministry of Health, Czech Republic, no. 00023736, by the Academy of Sciences, Czech Republic no. P205/12/G118 and NV18-08-00149, by ERDF OPPK CZ.2.16/3.1.00/24001 and...
Source: Blood - November 21, 2018 Category: Hematology Authors: Štikarova, J., Suttnar, J., Sovova, Z., Ceznerova, E., Novak, J., Louzil, J., Kotlin, R., Roselova, P., Kaufmanova, J., Chrastinova, L., Hlavackova, A., Majek, P., Kucerka, O., Hajsl, M., Maly, M., Dyr, J. E. Tags: 321. Blood Coagulation and Fibrinolytic Factors Source Type: research

Efficacy and Safety of Direct Oral Factor Xa Inhibitors in 795 Morbidly Obese Patients
Conclusions: Our study is the largest study examining morbidly obese patients on DOACS and provides further evidence of comparable efficacy and safety of the direct oral anti-Xa inhibitors, compared to warfarin, in morbidly obese patients with AF and VTE.DisclosuresKushnir: Janssen: Research Funding. Billett: Bayer: Consultancy; Janssen: Research Funding.
Source: Blood - November 21, 2018 Category: Hematology Authors: Kushnir, M., Choi, Y., Eisenberg, R., Rao, D., Tolu, S., Gao, J., Mowrey, W., Billett, H. H. Tags: 332. Antithrombotic Therapy: Management of Challenging Patients and Scenarios Source Type: research

Efficacy and Safety of Prothrombin Complex Concentrate (PCC) for Direct Oral Anticoagulant Reversal: A Single Institutional Experience
ConclusionIn this cohort, 80% of pts demonstrated clinical stability/improvement of hemorrhage. All pts were able to undergo surgical procedures; however 17% required additional FFP to achieve hemostasis. Thromboembolism occurred in 12% of patients. No deaths could definitely be attributed to 4PCC; however, 30% of deaths could be attributable to the underlying hemorrhagic episode.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Sridharan, M., Ou, N. N., Oyen, L. J., Ashrani, A. A., McBane, R. D., Pruthi, R. K. Tags: 332. Antithrombotic Therapy: Poster II Source Type: research

Predictors of Adverse Outcome in Sickle Cell Disease Patients from Oman
Conclusion: Multi-organ failure was mostly associated with the terminal events relating to mortality. Although sepsis played a major role as a cause of death in association with multiorgan failure, low hemoglobin, and low platelet counts with increased WBC counts along with elevated LDH and CRP played a significant role in the terminal event in this cohort of SCD patients.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Alkindi, S., AlJadidi, S., Al Adawi, S., Pathare, A. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science Source Type: research

Cerebral Infarcts and Cerebrovascular Disease in Neurologically Intact Tanzanian Children with Sickle Cell Anaemia
Conclusions:The prevalence of SCI on MRI is high in children with SCA without neurological history living in Africa even when TCD CBFV is normal. Children and adolescents with all grades of vasculopathy on MRA are at higher risk of brain parenchymal injury. The lack of association between SCI and hemoglobin may be related to the relatively severe anemia in our African study. As MRA contrast depends on velocity of blood flowing in vessels, it is not surprising that Grade 1 turbulence on MRA appears to reflect high CBFV on TCD. Importantly, high TCD velocity and Grade 1 turbulence may reflect the potentially reversible early...
Source: Blood - November 21, 2018 Category: Hematology Authors: Mwakatika, M. M., Saunders, D. E., Makani, J., Kirkham, F. J. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster I Source Type: research

A Genetic Variant in GOLGB1 Affects Golgi Function and Stroke Risk in Patients with Sickle Cell Disease
Conclusions: Our study has shown that a coding variant in GOLGB1, identified as protective against risk of stroke in patients with SCD, has significant effects on Golgi function in SCD samples. We observed that having the GOLGB1 Y1212C variant resulted in more compact and less fragmented Golgi apparatus. Proteomic analysis showed that SCD patients with the GOLGB1 variant also had significantly lower levels of proteins involved in platelet activation and Golgi trafficking. Our findings suggest a novel role for the Golgi apparatus in controlling protein flux that modulates risk of stroke in SCD.DisclosuresNo relevant conflic...
Source: Blood - November 21, 2018 Category: Hematology Authors: Kotu, V., Dubrelle, J., Baker, J., Jenson, K., Flanagan, J. M. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster III Source Type: research

Burden and Risk of Neurological and Cognitive Impairment in Pediatric Sickle Cell Anemia in Uganda (BRAIN SAFE): Final Results of the Cross-Sectional Analysis
Conclusions: These data demonstrate early, frequent neurovascular pathology for each outcome tested. Abnormal neurocognitive test results significantly increased with age or non-normal TCD. Our findings provide a baseline for longitudinal assessment and incentive for intervention. Enhanced research capacity was gained for junior faculty, trainees and staff, in areas of the conduct of brain research and focus on pediatric SCD, TCD performance and neuro-epidemiology.Funding: 1R21HD089791 (PIs: Idro, Green)DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Green, N. S., Munube, D., Bangirana, P., Opoka, R., Kayongo, E., Mupere, E., Buluma, L. R., Kebirungi, B., Sekibira, R., Kiguli, S., Kawooya, M., Lubowa, S. K., Elkind, M. S. V., Li, G., LaRussa, P., Idro, R. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research

Brain Magnetic Resonance Imaging and Angiography Findings in Ugandan Children with Sickle Cell Anemia; A Cross Sectional Study
ConclusionsBrain injury in Uganda children with SCA begins early in childhood and becomes a common finding during later childhood. Early screening for stroke and intervention therapy is warranted to prevent sickle brain vasculopathy initiated early after diagnosis. The risk factors for such early brain injury should be investigated.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Idro, R., Green, N. S., Munube, D., Buluma, L. R., Kebirungi, B., Opoka, R., Bangirana, P., Mupere, E., Kayongo, E., Sekibira, R., Kasirye, P., Lubowa, S. K., Kawooya, M., LaRussa, P., Minja, F. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research