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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

Tobacco Use in the Myeloproliferative Neoplasms: Patient Behavior, Opinions, and Care
ConclusionsMPN patients with current or previous tobacco use demonstrate significantly higher symptom burden than non-smoking counterparts. In terms of patient care, less than half of patients who are current or previous smokers recall having a physician discuss their smoking habits with them. These results highlight the need for enhanced MPN patient counseling by health care providers, both regarding the risks of smoking and available methods to aid cessation.DisclosuresScherber: Orphan Pharmaceuticals: Honoraria; Incyte: Consultancy. Dueck: Phytogine: Employment; Pfizer: Honoraria; Bayer: Employment. Palmer: Novartis: Re...
Source: Blood - November 21, 2018 Category: Hematology Authors: Scherber, R. M., Geyer, H. L., Mazza, G., Langlais, B. T., Dueck, A. C., Palmer, J., Padrnos, L., Fleischman, A., Mesa, R. A. Tags: 902. Health Services Research-Malignant Diseases 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

Interval decline in hemoglobin A is associated with annual clinical event rate in sickle cell anemia patients receiving maintenance apheresis RBC exchange.
CONCLUSIONS: Patients exhibiting greater daily HbA decrement were more likely to have multiple emergency department visits or admissions for sickling crises. Modulating HbA decrement may merit study as an intermediate metric for interventions to improve outcomes in hemoglobin SS disease. PMID: 31161685 [PubMed - as supplied by publisher]
Source: Transfusion - June 2, 2019 Category: Hematology Authors: Kamyszek RW, Raval JS, Srinivasan AJ, Ansari AK, Evans BA, Rollins-Raval MA, Poisson JL, Shah NR, Welsby IJ Tags: Transfusion Source Type: research

Curing hemoglobinopathies: challenges and advances of conventional and new gene therapy approaches.
Inherited hemoglobin disorders, including beta-thalassemia (BT) and sickle-cell disease (SCD) are the most common monogenic diseases worldwide, with a global carrier frequency of over 5%. With migration they are becoming more common worldwide, making their management and care an increasing concern for health care systems.BT is characterized by an imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and compensatory haemopoietic expansion. Globally, there are over 25,000 births each year with transfusion-dependent thalassemia (TDT). The current available treatment f...
Source: Mediterranean Journal of Hematology and Infectious Diseases - October 30, 2019 Category: Hematology Source Type: research

Sickle cell disease as a vascular disorder.
Authors: Ofori-Acquah SF Abstract Introduction: In sickle cell disease (SCD), hemoglobin S (HbS) red blood cells (RBCs) are characteristically deformed and inflexible. Often breaking down in the circulation, they exhibit increased adhesive properties with the endothelium and activated neutrophils and platelets, increasing the risk of occlusion of the microcirculation. SCD is categorized into two sub-phenotypes: hyperhemolytic, associated with priapism, leg ulcers, pulmonary hypertension, and stroke, and high hemoglobin/viscosity, which may promote vaso-occlusion-associated pain, acute chest syndrome, and osteonecro...
Source: Expert Review of Hematology - May 6, 2020 Category: Hematology Tags: Expert Rev Hematol Source Type: research

Allogenic Hematopoietic Stem Cell Transplantation in Sickle Cell Disease
Sickle cell disease (SCD) is one of the most common monogenic disorders worldwide and affects approximately 100,000 people in the United States alone. SCD can cause numerous complications, including anemia, pain, stroke, and organ failure, which can lead to death. Although there are a few disease-modifying treatments available to patients with SCD, the only current curative option is a hematopoietic stem cell transplant (HSCT). In this review, we will discuss the different approaches to allogeneic HSCT in the treatment of SCD and the outcomes of these approaches.
Source: Transfusion and Apheresis Science - January 10, 2021 Category: Hematology Authors: Dana K. Furstenau, John F. Tisdale Source Type: research

Clinical Characteristics of Pediatric Patients with Congenital Erythrocytosis: A Single-Center Study
AbstractAlthough congenital erythrocytosis (CE), an inherited disorder, impairs pediatric quality of life, physicians often overlook high hemoglobin (Hgb) levels and its symptoms due to lack of knowledge of age-adjusted pediatric Hgb levels and CE ’s rarity. In a retrospective, single-center study, data from hospital records of pediatric patients diagnosed with CE were evaluated. Twenty-six patients from 25 families (80.8% male) had been diagnosed with CE in 20 years, at a mean age of 14.9 ± 2.8 years (8.3–17.8) and with a mean Hgb level of 17.36 ± 1.44 g/dL (14.63–22.1). No serum erythropoietin levels e...
Source: Indian Journal of Hematology and Blood Transfusion - August 25, 2021 Category: Hematology Source Type: research

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