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Bleeding Related Episodes, Thrombotic Events and Platelet Counts Among Immune Thrombocytopenia Patients Receiving Second Line Therapy
CONCLUSIONS: This retrospective real world evidence study compares mean platelet response and burden of both BREs and TEs in ITP patients treated with different second-line treatments. Although there were significant differences in mean platelet counts achieved with the second-line treatments, TEs were observed with similar incidence across all treatments. Patients who received splenectomy had the highest mean platelet counts and also the highest proportion of patients who experienced TEs, though differences in TEs did not reach statistical significance. Incidence proportion of patients with BREs was lower in patients trea...
Source: Blood - November 21, 2018 Category: Hematology Authors: Said, Q., Lal, L. S., Andrade, K., Nezami, B., Graves, J. A., Roy, A., Cuker, A. Tags: 311. Disorders of Platelet Number or Function: Poster II 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

Validation of Sickle Cell Disease Severity Score in a Cohort of Hemoglobin SC Disease Patients
Conclusion: Despite having been derived from a SCD population that was 26% HbSC, the study was unable to validate the SSS within the cohort of HbSC patients. This may reflect the differences in patient population and/or therapeutic intervention between this cohort and the CSSCD cohort used in the construction of the SSS calculator. While SSS was found to correlate with 3 discrete markers of disease morbidity (TRJV, CVA, creatinine clearance), it appears that a new scoring system is required to accurately predict clinical mortality and morbidity in contemporary cohorts of adult HbSC disease patients.DisclosuresNo relevant c...
Source: Blood - November 21, 2018 Category: Hematology Authors: Rosart, R. M., Pestrin, O. A., Tomlinson, G. A., Ward, R., Pendergrast, J., Binding, A., Kuo, K. H. M. Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster I Source Type: research

Impact of Immune Thrombocytopenia in Patients Admitted with Coronary Artery Disease: Results from National Inpatient Sample 2009-2011
Conclusion: Our study shows that in patients with coronary artery disease-associated ITP, adjusted LOS and mortality was significantly higher compared to patients without ITP.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Aryal, M., Basnet, S., Dhital, R., Gosain, R., Donato, A. Tags: 903. Outcomes Research-Non-Malignant Hematology Source Type: research

Reassessing the Need for Pre-Operative Transfusions in Sickle Cell Disease Patients with a High Baseline Hemoglobin
Conclusion: Patients with SCD and a baseline hemoglobin ≥90 g/L who receive a pre-operative top-up transfusion have an increased risk of post-operative complications compared to those who are not transfused. In low and medium risk surgeries, a policy of withholding transfusions for such patients may be considered. Prospective studies validating these findings are needed.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Tole, S., Yan, A. P., Wagner, A., Bair, L., Tang, K., Kirby-Allen, M. A., Simpson, E., Williams, S. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research