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Practice Patterns in the Use of MRI/MRA and Chronic Transfusion Therapy for Monitoring and Treatment of Stroke in Pediatric Patients with Sickle Cell Anemia
Conclusions: The results of the DISPLACE practice patterns survey suggested nearly universal adoption of CRCT for children with abnormal TCD and prior overt stroke across sites. The use of MRI/MRA in detecting potential cerebrovascular abnormalities in the absence of clinical neurologic symptoms and for monitoring patients on CRCT was much more variable, which likely reflects the minimal amount of guidance for the use of MRI/MRA in SCA. Notably, a number of sites were using MRI/MRA to screen pediatric patients for cerebrovascular abnormalities and to monitor patients on CRCT. The variability in responses for MRI/MRA highli...
Source: Blood - November 21, 2018 Category: Hematology Authors: Schlenz, A. M., Mueller, M., Phillips, S., Melvin, C., Adams, R. J., Kanter, J. Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III Source Type: research

Type 2 Diabetes Mellitus in Patients with Sickle Cell Disease: A Population-Based Longitudinal Analysis of Three Cohorts
ConclusionWe present evidence describing the prevalence of T2DM in patients with SCD both in a commercially-insured population and from an institution-based clinical cohort. These findings were similar to a general African American population with an increasing trend in T2DM over recent years. These trends support the routine screening for T2DM in patients with SCD, especially those of older age and with presence of comorbid hypertension and/or dyslipidemia.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Zhou, J., Han, J., Nutescu, E. A., Galanter, W., Walton, S. M., Gordeuk, V. R., Saraf, S. L., Srisuwananukorn, A., Calip, G. S. Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster III Source Type: research

Outcomes of Hospitalization for Stem Cell Transplant in Sickle Cell Disease: Are We There Yet?
Conclusions: The rate of inpatient mortality with SCT in sickle cell disease is lower than the overall inpatient mortality rate for allogeneic SCT (7%; Godara et al bbmt 2018), indicating a favorable outcome for these patients. Infections do occur commonly during the course of hospitalization, especially in association with GVHD. Length of stay is adversely impacted by occurrence of GVHD, bacterial sepsis, C.difficile infection and viral infections. While we are limited by duration of follow up in our study, these patterns suggest some essential modifiers for inpatient morbidity and mortality, therefore require validation ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Godara, A., Siddiqui, N., Afzal, A., Khan, M., Yared, J., Kansagra, A., Dahiya, S. Tags: 732. Clinical Allogeneic Transplantation: Results: Poster III Source Type: research

Sudden Death in Sickle Cell Disease: An Assessment of Risk Factors
ConclusionOur results suggest that ACS and priapism may be indicative of a high risk of sudden death in patients with SCD. Our data are preliminary and need to be further investigated using a larger cohort of patients, as well as exploring the prevalence of these factors in SCD patients who are not yet deceased.DisclosuresAchebe: Bluebird Bio: Consultancy; AMAG Pharmaceuticals: Other: Advisory Board; Luitpold pharmaceuticals: Consultancy.
Source: Blood - November 21, 2018 Category: Hematology Authors: Nze, C., Fortin, B. M., Freedman, R., Puligandla, M., Neuberg, D. S., Mandell, E., Achebe, M. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical Source Type: research

Hemolytic, Vaso-Occlusive and Renal Complications of SCD: Report from the Central Missouri Cohort
ConclusionsThis is the first report describing prevalence of SCD-related complications in the MU-SCD Cohort. We identified this population to have an increasing frequency of hemolytic complications and sickle cell nephropathy with advancing age. Onset of persistent proteinuria occurred in the second decade of life, followed by renal insufficiency or end stage renal disease in subsequent decades. As previously demonstrated in the Cooperative Study of Sickle Cell Disease and the Jamaican SCD Cohort study, renal insufficiency was a significant risk factor for early mortality. Further studies are required for identification of...
Source: Blood - November 21, 2018 Category: Hematology Authors: Nolan, L. W., Yoshida, Y., Coberly, E., Sathi, B. K. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster I Source Type: research

Comprehensive Sickle Cell Disease Management Is Associated with Superior Grade Attainment and High School Graduation Rates
Discussion: In this cohort, a high percentage of patients (86.7%) graduated or were on track to graduate from high school. In the United States, approximately 90% of patients with SCD are African American. All patients analyzed here were African American. With this in mind, ACHSCDP patients' graduation rate compares favorably to estimated graduation rates across the US general population (84%), the U.S. African America population (76%), the U.S. sickle cell patient population (71%), the Ohio general population (84%), and the Ohio African American population (68%).The higher graduation rate among children at the ACHSCDP is ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Schulte, K., Mikofalvy, K., Beck, L., Bodas, P. V. Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III Source Type: research

Results from the Displace Consortium: Practice Patterns on the Use of Transcranial Doppler Screening for Risk of Stroke in Children with Sickle Cell Anemia
Conclusions: Nearly all DISPLACE sites order TCD screening annually, as recommended in the guidelines, with some ordering screening more frequently. A few sites did not report initiation of CRCT per STOP protocol for abnormal TCD results; however, over half of the sites reported following up with an MRI/MRA, which may suggest evaluating for vasculopathy prior to CRCT. Some sites reported beginning HU therapy for abnormal results; this may reflect consideration of patients for whom CRCT is not possible, but data were not collected for confirmation. Interestingly, results suggest a reliance on MRI/MRA since sites commonly re...
Source: Blood - November 21, 2018 Category: Hematology Authors: Phillips, S., Mueller, M., Schlenz, A. M., Melvin, C., Adams, R. J., Kanter, J. Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III Source Type: research

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