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Immunophenotypic Parameters and RBC Alloimmunization in Children with Sickle Cell Disease on Chronic Transfusion
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Source: American Journal of Hematology - September 1, 2015 Category: Hematology Authors: Robert S. Nickel, John T. Horan, Ross M. Fasano, Erin Meyer, Cassandra D. Josephson, Anne M. Winkler, Marianne E. M. Yee, Leslie S. Kean, Jeanne E. Hendrickson Tags: Research Article Source Type: research

Organ iron accumulation in chronically transfused children with sickle cell anaemia: baseline results from the TWiTCH trial
We report the baseline findings of abdominal organ iron burden in 121 participants. At enrollment, patients were young (9·8 ± 2·9 years), predominantly female (60:40), and previously treated with transfusions (4·1 ± 2·4 years) and iron chelation (3·1 ± 2·1 years). Liver iron concentration (LIC; 9·0 ± 6·6 mg/g dry weight) and serum ferritin were moderately elevated (2696 ± 1678 μg/l), but transferrin was incompletely saturated (47·2 ± 23·6%). Spleen R2* was 509 ± 399 Hz (splenic iron ~13·9 mg/g) and correlated with LIC (r2 = 0·14, P = 0·0008). Pancreas R2* was increased in 38·3...
Source: British Journal of Haematology - November 2, 2015 Category: Hematology Authors: John C. Wood, Alan R. Cohen, Sara L. Pressel, Banu Aygun, Hamayun Imran, Lori Luchtman‐Jones, Alexis A. Thompson, Beng Fuh, William H. Schultz, Barry R. Davis, Russell E. Ware, Tags: Research Paper Source Type: research

Effectiveness of red blood cell exchange, partial manual exchange, and simple transfusion concurrently with iron chelation therapy in reducing iron overload in chronically transfused sickle cell anemia patients.
CONCLUSION: We recommend RCE plus chelation as an effective method for reducing iron overload, while maintaining HbS at 30% to 35%. PMID: 26997031 [PubMed - as supplied by publisher]
Source: Transfusion - March 19, 2016 Category: Hematology Authors: Fasano RM, Leong T, Kaushal M, Sagiv E, Luban NL, Meier ER Tags: Transfusion Source Type: research

Management of Patients with Sickle Cell Disease Using Transfusion Therapy
Publication date: June 2016 Source:Hematology/Oncology Clinics of North America, Volume 30, Issue 3 Author(s): Stella T. Chou, Ross M. FasanoTeaser Red blood cell (RBC) transfusion therapy is a key component of comprehensive management of patients with sickle cell disease (SCD) and has increased over time as a means of primary and secondary stroke prevention. RBC transfusions also prove to be lifesaving for many acute sickle cell–related complications. Although episodic and chronic transfusion therapy has significantly improved the morbidity and mortality of patients with SCD, transfusions are not without adverse eff...
Source: Hematology Oncology Clinics of North America - April 23, 2016 Category: Hematology Source Type: research

Chronic transfusion therapy for stroke in sickle cell disease
Source: Journal of Clinical Apheresis - February 5, 2017 Category: Hematology Authors: Michael R. DeBaun, Keith Quirolo Tags: LETTER TO THE EDITOR 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

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

Red blood cell mechanical sensitivity improves in patients with sickle cell disease undergoing long-term transfusion after prolonged, subhemolytic shear exposure.
CONCLUSION: We found that long-term transfusion therapy normalizes the MS threshold above which RBC subhemolytic damage occurs after prolonged shear exposure in SCD. An important and novel finding in transfused patients with SCD was the improvement in RBC deformability in response to prolonged shear exposure over the physiologic range. PMID: 30325033 [PubMed - as supplied by publisher]
Source: Transfusion - October 16, 2018 Category: Hematology Authors: Simmonds MJ, Suriany S, Ponce D, Detterich JA Tags: Transfusion Source Type: research

Splenectomy to Optimize Hemoglobin S Control in Children With Sickle Cell Anemia on Chronic Transfusion Therapy for Stroke Prevention
Chronic transfusion therapy with the goal of maintaining a hemoglobin (Hb) S
Source: Journal of Pediatric Hematology Oncology - February 21, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Red cell transfusion and alloimmunization in sickle cell disease
Haematologica. 2021 Apr 1. doi: 10.3324/haematol.2020.270546. Online ahead of print.ABSTRACTRed cell transfusion remains a critical component of care for acute and chronic complications of sickle cell disease. Randomized clinical trials demonstrated the benefits of transfusion therapy for prevention of primary and secondary strokes and postoperative acute chest syndrome. Transfusion for splenic sequestration, acute chest syndrome, and acute stroke are guided by expert consensus recommendations. Despite overall improvements in blood inventory safety, adverse effects of transfusion are prevalent among patients with sickle ce...
Source: Haematologica - April 1, 2021 Category: Hematology Authors: Grace E Linder Stella T Chou Source Type: research

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