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Total 18 results found since Jan 2013.
Mast Cells Contribute to Brain Microvascular Permeability in Sickle Cell Disease
We examined the ability of mast cells to stimulate P-selectin expression and BBB permeability via ER stress in a sickle microenvironment.We isolated MCs from HbAA-BERK and HbSS-BERK, control and sickle mice, respectively; incubated them in vitro and collected mast cell conditioned media (MCCM) from HbAA MCs and HbSS MCs. Normal mouse brain microvascular endothelial cells (mBMECs) were treated with unconditioned MCCM, HbAA MCCM, or HbSS MCCM to examine the effect of mast cell activation on endothelium. We observed increased mast cell activity in HbSS mice evinced by significantlyhigher plasma and skin histamine levels, comp...
Source: Blood - November 21, 2018 Category: Hematology Authors: Mittal, A. M., Tran, H., Sagi, V., Nguyen, A., Luk, K., Nguyen, J., Lei, J., Gupta, K. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Sickle Cell Disease-Role of Coagulation and Inflammation in Pathophysiology Source Type: research
Transfusion Therapy in a Multi-Ethnic Sickle Cell Population Real-World Practice. a Preliminary Data Analysis of Multicentre Survey
Conclusion. The transfusional approach is similar in HbSS, b°-thal/HbS and b+-thal/HbS patients with similar indications, prevalently VOCs and anemia. The significant higher age in Caucasian cohort and the consequent long term follow up could be the cause of variable therapeutic approach observed, however Hydroxycarbamide seemed to be the therapy more frequently used and finally suggested to manage chronic manifestations.Figure.DisclosuresOriga: Apopharma: Honoraria; Novartis: Honoraria; Bluebird Bio: Consultancy; Cerus Corporation: Research Funding. Forni: Apopharma: Other: DSM Board; Celgene: Research Funding; Novart...
Source: Blood - November 21, 2018 Category: Hematology Authors: Graziadei, G., Sainati, L., Bonomo, P., Venturelli, D., Masera, N., Casale, M., Vassanelli, A., Lodi, G., Piel, F. B., Voi, V., De Franceschi, L., Rigano, P., Quota, A., Notarangelo, L. D., Russo, G., Rosso, R., Allo, M., D'Ascola, D., Facchini, E., Macch Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research
Characterization and treatment of congenital thrombotic thrombocytopenic purpura
In conclusion, prespacer mutations are associated with earlier development of cTTP symptoms. Prophylactic ADAMTS13 replacement decreases the risk of end-organ damage such as ischemic stroke and resolved previously unrecognized symptoms in patients with nonovert disease.
Source: Blood - April 10, 2019 Category: Hematology Authors: Alwan, F., Vendramin, C., Liesner, R., Clark, A., Lester, W., Dutt, T., Thomas, W., Gooding, R., Biss, T., Watson, H. G., Cooper, N., Rayment, R., Cranfield, T., van Veen, J. J., Hill, Q. A., Davis, S., Motwani, J., Bhatnagar, N., Priddee, N., David, M., Tags: Thrombocytopenia, Platelets and Thrombopoiesis, Clinical Trials and Observations Source Type: research
Results from Part A of the Hemoglobin Oxygen Affinity Modulation to Inhibit HbS Polymerization (HOPE) Trial (GBT440-031), a Placebo-Controlled Randomized Study Evaluating Voxelotor (GBT440) in Adults and Adolescents with Sickle Cell Disease
Conclusions: Data from Part A of the HOPE study demonstrate that treatment with voxelotor resulted in a dose-dependent increase in Hb with a large proportion of patients achieving Hb >1 g/dL improvement from baseline compared with placebo at 12 weeks. In addition, there was a dose-dependent decrease in hemolysis markers. Voxelotor was generally well tolerated at both doses. Hemolytic anemia of SCD has severe and life-threatening consequences and presents an unmet medical need. Voxelotor has potential to ameliorate complications of anemia associated with SCD.DisclosuresVichinsky: bluebird bio: Membership on an entity's B...
Source: Blood - November 21, 2018 Category: Hematology Authors: Vichinsky, E., Hoppe, C., Howard, J., Ataga, K. I., Nduba, V., El-Beshlawy, A., Diuguid, D. L., Al-Kindi, S., Brown, C., Hassab, H., Telfer, P., Tsitsikas, D. A., Unal, S., Kanter, J., Abboud, M. R., Gordeuk, V. R., Lehrer-Graiwer, J., Sherman, C., Tonda, Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Novel or Improved Approaches To Treating Sickle Cell Disease Source Type: research
Clinical and Laboratory Benefits of Early Initiation of Hydroxyurea with Pharmacokinetic Guided Dosing for Young Children with Sickle Cell Anemia
Conclusions: Hydroxyurea initiation at an early age using PK-guided dosing provides significant clinical benefits for young children with sickle cell anemia. These TREAT study data suggest that initiating hydroxyurea around one year of life using a personalized dosing strategy can provide better clinical and laboratory benefits than starting at the conventional 20 mg/kg/day weight-based dose. Very high HbF levels are observed at modest and well-tolerated doses of hydroxyurea, perhaps because treatment was initiated before the process of HbF inactivation is complete. Continued long-term follow-up of these patients will dete...
Source: Blood - November 21, 2018 Category: Hematology Authors: McGann, P. T., Niss, O., Dong, M., Marahatta, A., Mizuno, T., Kalinyak, K., Kalfa, T. A., Malik, P., Quinn, C. T., Ware, R. E., Vinks, A. A. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Novel or Improved Approaches To Treating Sickle Cell Disease Source Type: research
Parenteral L-Arginine Improves Mitochondrial Function in Children with Sickle Cell Disease Admitted for Vaso-Occlusive Pain Episodes
Conclusion: These data demonstrate for the first time that Arg supplementation increases mitochondrial activity and decreases oxidative stress in children with SCD and VOE. Our prior study showed that complex V inhibition leads to increased mitochondrial oxidant production in platelets from SCD patients. The improvement in complex V function & decreased oxidative markers observed with Arg treatment are consistent with Arg-induced decrease in mitochondrial oxidant generation. However, Arg treatment potentially regulates other oxidant sources and could decrease overall oxidative stress in SCD patients. Arg therapy has be...
Source: Blood - November 21, 2018 Category: Hematology Authors: Shiva, S., Brown, L. A., Wang, Y., Dampier, C., Watt, A., Tripathi, V., Bhutta, A., Kumari, P., Figueroa, J., Zmitrovich, A., Morris, C. R. Tags: 114. Hemoglobinopathies, Excluding Thalassemia Clinical: Novel or Improved Approaches To Treating Sickle Cell Disease Source Type: research
Current Results of Lentiglobin Gene Therapy in Patients with Severe Sickle Cell Disease Treated Under a Refined Protocol in the Phase 1 Hgb-206 Study
Backgroundβ-globin gene transfer has the potential for substantial clinical benefit in patients with sickle cell disease (SCD). LentiGlobin Drug Product (DP) contains autologous CD34+ hematopoietic stem cells (HSCs) transduced with the BB305 lentiviral vector (LVV), encoding β-globin with an anti-sickling substitution (T87Q). The safety and efficacy of LentiGlobin gene therapy is being evaluated in the ongoing Phase 1 HGB-206 study (NCT02140554). Results in the initial 7 patients treated with LentiGlobin DP from steady state bone marrow harvested (BMH) HSCs using original DP manufacturing process (Group A) demons...
Source: Blood - November 21, 2018 Category: Hematology Authors: Tisdale, J. F., Kanter, J., Mapara, M. Y., Kwiatkowski, J. L., Krishnamurti, L., Schmidt, M., Miller, A. L., Pierciey, F. J., Shi, W., Ribeil, J.-A., Asmal, M., Thompson, A. A., Walters, M. C. Tags: 801. Gene Therapy and Transfer: Gene Therapy for Blood Cell Disorders Source Type: research
Regulatory Genetic Variation at the S100B Gene Associates with Vaso-Occlusive Manifestations in Sickle Cell Disease
In sickle cell disease (SCD) polymerization of hemoglobin S under deoxygenated conditions causes vaso-occlusion, which can manifest as acute pain crisis and progressive bone/organ damage. Molecular studies have attributed vaso-occlusion to elevated vascular adhesion and inflammatory responses, whereas the genetic regulation has only recently been assessed.Genomic DNA isolated from peripheral blood mononuclear cells (PBMCs) was hybridized to Illumina Human 610-Quad SNP array for the PUSH and Walk-PHaSST cohorts and to Affrymetrix SNP 6.0 array for the Howard SCD expression cohort. Single nucleotide polymorphisms (SNPs) for ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Zhang, X., Zhang, W., Saraf, S. L., Nekhai, S., Gladwin, M. T., Machado, R., Gordeuk, V. R. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster I 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
Mean Platelet Volume Predicts Mortality in Sickle Cell Disease
Conclusion:MPV is an independent biomarker predicting disease severity and probability of death in patients with sickle cell disease with a strong correlation especially in pediatric age group. Although there was no statistical significance across age groups, the variation of MPV for each patient with age needs to be studied for better understanding. Hydroxyurea a known disease-ameliorating agent is associated with lower MPV values in pediatric age group. This effect is independent of the levels of fetal hemoglobin and may be due to anti-inflammatory effect of hydroxyurea or decreased platelet consumption.Figure.Disclosure...
Source: Blood - November 21, 2018 Category: Hematology Authors: Thavamani, A., Ramanathan, R., Puliyel, M. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II 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
Quality of Care Indicators in Patients with Sickle Cell Disease (SCD): Influenza Vaccination and Routine Eye Examination Are Associated with Outpatient Utilization but Not Acute Hospital Care, Whereas Comorbidity Indexes Are Strongly Associated with Both
In conclusion, outpatient-based candidate quality indicators of care examined (influenza vaccination and eye examination), were associated with increased outpatient utilization, but not acute visits or hospitalizations. Co-morbidity indexes have significant confounding effects on outpatient and hospital utilization. Administrative data based quality indicators of care such as influenza vaccination, as well as SCD specific comorbidity indexes warrant further studies as these may help better understand optimal allocation of health care resources for patients with SCD.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Adamkiewicz, T., Baltrus, P., Li, C., Carter-Wicker, K., Gaglioti, A. Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster II Source Type: research
Defining Sickle Cell Disease Severity Among Adults Hospitalized with Vaso-Occlusive Crisis
Conclusions: The high severity scores among this cohort supports the validity of employing a medical history checklist as a measure of disease severity, reflecting the cumulative end organ damage due to SCD and near universal need for daily pain medication among patients requiring inpatient admission for VOC. Two-thirds of patients were categorized as having high disease severity, driven primarily by daily pain medication use prior to hospitalization and pulmonary complications, consistent with a known subgroup of adults with SCD-related complications who are more likely to have lower health-related quality of life and acc...
Source: Blood - November 21, 2018 Category: Hematology Authors: Esham, K. S., Rodday, A. M., Savidge, N., Mao, D., Weidner, R. A., Parsons, S. K. Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster II Source Type: research
Monoclonal Gammopathy of Undetermined Significance - Patient Characteristics and Referral Patterns
ConclusionMGUS is often incidentally detected as part of a work up for other medical conditions, and our results reveal that there is a variety of reasons for which monoclonal testing is performed. With recent developments in our understanding of the significance of monoclonal gammopathy and its association with certain renal and organ damage (Fermand et al., 2018; Leung et al., 2012), there may be a change in how the paraproteinemia investigations are utilized by clinicians in different disciplines. It will be important to recognize and establish appropriate indications for testing. Furthermore, MGUS patients present with...
Source: Blood - November 21, 2018 Category: Hematology Authors: Lee, H., Street, L., Tay, J., Grossman, J., Thaell, J. F., Goodyear, D., McCulloch, S., Duggan, P., Neri, P., Jimenez-Zepeda, V. Tags: 651. Myeloma: Biology and Pathophysiology, excluding Therapy: 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
