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Specialty: Hematology
Condition: Anemia

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Total 197 results found since Jan 2013.

Unwinding the path from anemia to stroke
Source: Blood - March 1, 2018 Category: Hematology Authors: Wood, J. C. Tags: Free Research Articles COMMENTS 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

Children with sickle cell anemia with normal TCD and without silent infarcts have a low incidence of new strokes
This article is protected by copyright. All rights reserved.
Source: American Journal of Hematology - March 9, 2018 Category: Hematology Authors: Lori C. Jordan, Dionna O. Roberts Williams, Mark J. Rodeghier, Brittany Covert, Maria R. Ponisio, James F. Casella, Robert C. McKinstry, Michael J. Noetzel, Fenella J. Kirkham, Emily R. Meier, Beng Fuh, Melissa McNaull, Sharada Sarnaik, Suvankar Majumdar, Tags: Research Article Source Type: research

A model-based cost-effectiveness analysis of Patient Blood Management.
DISCUSSION: Our results indicate that PBM may be associated with fewer adverse clinical outcomes compared to control management and may, thereby, be cost-effective. PMID: 29517965 [PubMed - as supplied by publisher]
Source: Blood Transfusion - February 16, 2018 Category: Hematology Authors: Kleinerüschkamp A, Meybohm P, Straub N, Zacharowski K, Choorapoikayil S Tags: Blood Transfus Source Type: research

Proteomic analysis of plasma from children with sickle cell anemia and silent cerebral infarction.
Abstract Silent cerebral infarction is the commonest neurological abnormality in children with sickle cell anemia, affecting 30-40% 14 year olds. There are no known biomarkers to identify children with silent cerebral infarcts and the pathological basis is also unknown. We used an unbiased proteomic discovery approach to identify plasma proteins differing in concentration between children with and without silent cerebral infarcts. Clinical parameters and plasma samples were analysed from 51 children (mean age 11.8 years, range 6-18) with sickle cell anemia (HbSS). 19 children had silent cerebral infarcts and 32 no...
Source: Haematologica - March 15, 2018 Category: Hematology Authors: Tewari S, Renney G, Brewin J, Gardner K, Kirkham F, Inusa B, Barrett JE, Menzel S, Thein SL, Ward M, Rees DC Tags: Haematologica Source Type: research

Interleukin ‐6 G‐174C polymorphism predicts higher risk of stroke in sickle cell anaemia
British Journal of Haematology, EarlyView.
Source: British Journal of Haematology - May 23, 2017 Category: Hematology Source Type: research

Clinical manifestations of sickle cell disease in India: misconceptions and reality
Purpose of review In the past, milder clinical manifestations of sickle cell disease (SCD) have been described from India. However, recent data from some parts of India suggest that the severity of the disease can be compared to that of African phenotypes. This review therefore describes the varied clinical manifestation of SCD, the success of newborn screening programme, prenatal diagnosis and low dose hydroxyurea therapy in India. Recent findings The varied clinical manifestations such as anemia, vaso-occlusive crisis, acute chest syndrome, renal involvement, stroke and so on vary from one part of the country to the...
Source: Current Opinion in Hematology - April 5, 2018 Category: Hematology Tags: ERYTHROID SYSTEM AND ITS DISEASES: Edited by Narla Mohandas Source Type: research

Simple chronic transfusion therapy, a crucial therapeutic option for sickle cell disease, improves but does not normalize blood rheology: What should be our goals for transfusion therapy?
Abstract Sickle cell anemia is characterized by a mutation resulting in the formation of an abnormal beta-hemoglobin called hemoglobin S. Hemoglobin S polymerizes upon deoxygenation, causing impaired red blood cell deformability and increased blood viscosity at equivalent hematocrits. Thus, sickle cell disease is a hemorheologic disease that results in various pathologic processes involving multiple organ systems including the lungs, heart, kidneys and brain. Red blood cell mechanics and the perturbations on blood flow-endothelial interaction underlie much of the pathology found in sickle cell disease. Transfusion...
Source: Clinical Hemorheology and Microcirculation - April 5, 2018 Category: Hematology Authors: Detterich JA Tags: Clin Hemorheol Microcirc Source Type: research

A rationale for universal tranexamic acid in major joint arthroplasty: overall efficacy and impact of risk factors for transfusion.
CONCLUSIONS: Universal TXA was associated with a reduction of RBC transfusion, overall and in clinically relevant subgroups, strengthening the rationale for universal therapy. PMID: 30383292 [PubMed - as supplied by publisher]
Source: Transfusion - November 1, 2018 Category: Hematology Authors: Pavenski K, Ward SE, Hare GMT, Freedman J, Pulendrarajah R, Pirani RA, Sheppard N, Vance C, White A, Lo N, Waddell JP, Ho A, Schemitsch EH, Kataoka M, Bogoch ER, Saini K, David Mazer C, Baker JE Tags: Transfusion Source Type: research

Reduced Cerebral Metabolic Rate of Oxygen in Adults with Sickle Cell Disease
Conclusion:We observed reduced CMRO2 in patients with SCD compared to healthy controls due to low OEF. A reduced CMRO2 could pose a risk for ischemia, despite high flow rate delivering oxygen, because of low OEF. This is supported by the fact that the silent cerebral infarcts are located in regions with the lowest CMRO2. We postulate that patients with SCD have a reduced capacity to increase the OEF in regions with inadequate CBF resulting in local ischemia and local infarction. The pathogenesis of the reduced OEF remains unclear but could be related to arteriovenous shunting whereby there is insufficient time for oxygen t...
Source: Blood - November 21, 2018 Category: Hematology Authors: Vaclavu, L., Petersen, E. T., VanBavel, E. T., Majoie, C. B., Nederveen, A. J., Biemond, B. J. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Organ Damage and Clinical Complications in Sickle Cell Disease Source Type: research

High Molecular Weight Kininogen Contributes to End-Organ Damage and Mortality in a Mouse Model of Sickle Cell Disease
Conclusions: These data indicate that HK deficiency attenuates chronic inflammation, kidney failure, and heart hypertrophy, and improves survival of sickle cell mice.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - November 21, 2018 Category: Hematology Authors: Sparkenbaugh, E., Wilson, K., Kasztan, M., Pollock, D. M., McCrae, K. R., Pawlinski, R. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Sickle Cell Disease-Role of Coagulation and Inflammation in Pathophysiology 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

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

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