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Central nervous system complications and management in sickle cell disease
With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ~11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA ...
Source: Blood - February 18, 2016 Category: Hematology Authors: DeBaun, M. R., Kirkham, F. J. Tags: Sickle Cell Disease, Free Research Articles, Red Cells, Iron, and Erythropoiesis, Review Articles, Review Series, Clinical Trials and Observations Source Type: research

Red cell exchange for patients with sickle cell disease: an international survey of current practices.
CONCLUSIONS: This survey solicited the current practice variations in RCE from a diverse range of practice sites. Many sites reported similar practice patterns and challenges but some variations emerged. To our knowledge, this survey represents the largest and most in-depth investigation of the use of RCE for patients with SCD, and could inform future studies in the field. PMID: 32583456 [PubMed - as supplied by publisher]
Source: Transfusion - June 23, 2020 Category: Hematology Authors: Karafin MS, Hendrickson JE, Kim HC, Kuliya-Gwarzo A, Pagano MB, Perumbeti A, Shi PA, Tanhehco YC, Webb J, Wong E, Eichbaum Q Tags: Transfusion 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

Indications for transfusion in the management of sickle cell disease
Hematology Am Soc Hematol Educ Program. 2021 Dec 10;2021(1):696-703. doi: 10.1182/hematology.2021000307.ABSTRACTThe transfusion of red blood cells (RBCs) is a crucial treatment for sickle cell disease (SCD). While often beneficial, the frequent use of transfusions is associated with numerous complications. Transfusions should be offered with specific guidelines in mind. Here we present updates to the indications for transfusion of RBCs in SCD. We review recent publications and include expert perspectives from hematology and transfusion medicine. For some clinical indications, such as ischemic stroke, the role of transfusio...
Source: Hematology ASH Education Program - December 10, 2021 Category: Hematology Authors: Hyojeong Han Lisa Hensch Ven ée N Tubman Source Type: research

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