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Total 11 results found since Jan 2013.
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
Home Based Primary Care for Patients with Sickle Cell Disease
Conclusion: Overall, home based primary care seems to be a promising alternative for pts with SCD. It had a significant impact on patient quality of care and may improve prescription adherence, but more data are needed to determine if it has an effect on healthcare utilization for pts with SCD.DisclosuresMoore: Ohio State University College of Medicine: Research Funding. Desai: FDA: Research Funding; Pfizer: Research Funding; University of Pittsburgh: Research Funding; Selexy/Novartis: Research Funding; NIH: Research Funding; Ironwood: Other: Adjudication Committee.
Source: Blood - November 21, 2018 Category: Hematology Authors: Moore, M. D., Schamess, A., Williams, N., Huang, Y., Menka, G., Desai, P. C. Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III Source Type: research
Outcome of Age-Adapted Approach to HLA-Identical Related Hematopoietic Stem Cell Transplantation in Severe Sickle Cell Disease: Saudi Experience
In this study, we reviewed the outcome of SCD patients who underwent transplant at our institution using standard protocols (NMA regimen in patients ≥14 years and myeloablative regimen in < 14 years) to address whether age remains a risk factor that influences HSCT outcome in SCD.Children (<14 years) with severe SCD received myeloablative conditioning using one of two regimens: first regimen was cyclophosphamide (Cy) 200mg/kg, busulfan (Bu) 16mg/kg, and thymoglobulin (ATG) 10mg/kg and recently we use thiotepa 8mg/kg, Bu 16mg/kg, and fludarabine (Flu) 160mg/m2. Bu pharmacokinetics was performed to target AUC of 900...
Source: Blood - November 21, 2018 Category: Hematology Authors: Alzahrani, M., Damlaj, M., Essa, M., Alahmari, B., Alaskar, A., Hejazi, A., Basher, E., Abujoub, R., Ghazi, S., Abuelgasim, K., Salama, H., Gmati, G., Alsultan, A. Tags: 732. Clinical Allogeneic Transplantation: Results: Poster II Source Type: research
Clinical Relevance of Clonal Hematopoiesis in the Oldest-Old Population: Analysis of the "Health and Anemia" Study
Conclusion. Clonal hematopoiesis was associated with reduced survival in an oldest-old population. Specific mutational profiles define different risks of developing MDS and inflammatory/vascular diseases. Non mutational factors, such as early changes in red blood cell indices, may improve the capability to identify patients at increased risk of developing myeloid cancers.DisclosuresMeggendorfer: MLL Munich Leukemia Laboratory: Employment. Bolli: Celgene: Honoraria. Vassiliou: KYMAB: Consultancy, Equity Ownership; Celgene: Research Funding. Kern: MLL Munich Leukemia Laboratory: Employment, Equity Ownership. Haferlach: MLL M...
Source: Blood - November 21, 2018 Category: Hematology Authors: Rossi, M., Meggendorfer, M., Zampini, M., Tettamanti, M., Riva, E., Saba, E., Manes, N., Milanesi, C., Marta, U., Morabito, L., Travaglino, E., Peano, C., Giulia, S., Asselta, R., Duga, S., Malik, K., Selmi, C., Civilini, E., Mandelli, S., Bolli, N., Vass Tags: 503. Clonal Hematopoiesis: Aging and Inflammation: Cause and consequence of clonal hematopoiesis Source Type: research
Measuring Health-Related Quality of Life in Sickle Cell Disease Patients Undergoing Automated Red Blood Cell Exchange in the USA, France and the UK
ConclusionSickle cell disease patients that require chronic blood transfusion experience better health-related quality of life when they are treated with automated red blood cell exchange versus simple transfusion. This observation is supported by the opinion of their treating physicians.DisclosuresDierick: Terumo BCT: Employment. Roig: Terumo BCT: Employment.
Source: Blood - November 21, 2018 Category: Hematology Authors: Dierick, K., Roig, J. Tags: 903. Outcomes Research-Non-Malignant Hematology Source Type: research
Acute Hemolysis Induces Pro-Angiogenic Molecule Production and Neovascularization In Vivo
Conclusions: Acute intravascular hemolysis was associated with rapid alterations in circulating angiogenic and inflammatory markers in mice. In association with this pro-angiogenic profile, in vivo neovascularization was accelerated in animals following hemolysis. These data suggest that hemolysis may be a significant stimulus for angiogenic processes, which in turn may contribute to some of the clinical complications of hemolytic diseases, including pulmonary hypertension, stroke and leg ulcers. Furthermore, the angiogenic process may represent a target for the development of therapeutic strategies in disorders characteri...
Source: Blood - November 21, 2018 Category: Hematology Authors: Gotardo, E. M. F., Chweih, H., Brito, P. L., Leonardo, F. C., Costa, R., Soares, R., Costa, F. F., Conran, N. Tags: 101. Red Cells and Erythropoiesis, Structure and Function, Metabolism, and Survival, Excluding Iron: Poster III Source Type: research
B0 Vs. Non-B0 Genotype: Differences in Non-Transfusion-Dependent Thalassemia Patients
Conclusions: Heart remodelling related to a high cardiac output state cardiomyopathy was more pronounced in patients with homozygous β°/β° genotype. Osteoporososis was significantly more frequent in patients with homozygous β°/β° genotype, treated for more than two-thirds with DFO therapy. These data support the knowledge of different phenotypic groups in the management of NTDT patients.TableDisclosuresPepe: Chiesi Farmaceutici S.p.A., ApoPharma Inc., and Bayer: Other: No profit support.
Source: Blood - November 21, 2018 Category: Hematology Authors: Pepe, A., Pistoia, L., Maddaloni, D., Grippo, T., Benni, M., Sardella, L., Sanna, M. G., Giugno, G., Guerrini, G., Maggio, A., Renne, S., Missere, M., Positano, V., Meloni, A. Tags: 112. Thalassemia and Globin Gene Regulation: Poster I Source Type: research
Role of Automated Red Cell Exchange in Acute and Chronic Complications of Sickle Cell Disease
Discussion. Our study shows that red cell exchange is an effective treatment modality for patients with sickle cell disease. It contributes to improvement in weight, increase in Hb and Hct and decrease in wbc, plt, HbS% and iron overload. It also decreases inpatient and ED admissions. The procedure is safe and tolerable with minimal complications. Long term studies are needed study the efficacy of this treatment modality and its contribution to improvement of quality of life and life expectancy in sickle cell disease patients.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Jain, S., Rock, A., Lopes, C., Saraf, S. L., Zhang, X., Gowhari, M., Molokie, R. E., Gordeuk, V. R., Campbell-lee, S. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster III Source Type: research
Silent infarcts in sickle cell disease occur in the border zone region and are associated with low cerebral blood flow
Silent cerebral infarcts (SCIs) are associated with cognitive impairment in sickle cell anemia (SCA). SCI risk factors include low hemoglobin and elevated systolic blood pressure; however, mechanisms underlying their development are unclear. Using the largest prospective study evaluating SCIs in pediatric SCA, we identified brain regions with increased SCI density. We tested the hypothesis that infarct density is greatest within regions in which cerebral blood flow is lowest, further restricting cerebral oxygen delivery in the setting of chronic anemia. Neuroradiology and neurology committees reached a consensus of SCIs in...
Source: Blood - October 18, 2018 Category: Hematology Authors: Ford, A. L., Ragan, D. K., Fellah, S., Binkley, M. M., Fields, M. E., Guilliams, K. P., An, H., Jordan, L. C., McKinstry, R. C., Lee, J.-M., DeBaun, M. R. Tags: Sickle Cell Disease, Free Research Articles, Red Cells, Iron, and Erythropoiesis, CME article Source Type: research
Red cell exchange transfusions lower cerebral blood flow and oxygen extraction fraction in pediatric sickle cell anemia
Blood transfusions are the mainstay of stroke prevention in pediatric sickle cell anemia (SCA), but the physiology conferring this benefit is unclear. Cerebral blood flow (CBF) and oxygen extraction fraction (OEF) are elevated in SCA, likely compensating for reduced arterial oxygen content (CaO2). We hypothesized that exchange transfusions would decrease CBF and OEF by increasing CaO2, thereby relieving cerebral oxygen metabolic stress. Twenty-one children with SCA receiving chronic transfusion therapy (CTT) underwent magnetic resonance imaging before and after exchange transfusions. Arterial spin labeling and asymmetric s...
Source: Blood - March 1, 2018 Category: Hematology Authors: Guilliams, K. P., Fields, M. E., Ragan, D. K., Eldeniz, C., Binkley, M. M., Chen, Y., Comiskey, L. S., Doctor, A., Hulbert, M. L., Shimony, J. S., Vo, K. D., McKinstry, R. C., An, H., Lee, J.-M., Ford, A. L. Tags: Pediatric Hematology, Sickle Cell Disease, Transfusion Medicine, Free Research Articles, Red Cells, Iron, and Erythropoiesis 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
