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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

Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial
The Stroke With Transfusions Changing to Hydroxyurea (SWiTCH) trial compared standard (transfusions/chelation) to alternative (hydroxyurea/phlebotomy) treatment to prevent recurrent stroke and manage iron overload in children chronically transfused over 7 years before enrollment. Standardized brain magnetic resonance imaging/magnetic resonance angiography (MRA) and transcranial Doppler (TCD) exams were performed at entry and exit, with a central blinded review. A novel MRA vasculopathy grading scale demonstrated frequent severe baseline left/right vessel stenosis (53%/41% ≥Grade 4); 31% had no vessel stenosis on either ...
Source: Blood - August 7, 2014 Category: Hematology Authors: Helton, K. J., Adams, R. J., Kesler, K. L., Lockhart, A., Aygun, B., Driscoll, C., Heeney, M. M., Jackson, S. M., Krishnamurti, L., Miller, S. T., Sarnaik, S. A., Schultz, W. H., Ware, R. E., for the SWiTCH Investigators Tags: Pediatric Hematology, Sickle Cell Disease, Free Research Articles, Red Cells, Iron, and Erythropoiesis, Vascular Biology, Clinical Trials and Observations Source Type: research