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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

Indications for RBC Exchange Transfusion in Patients with Sickle Cell Disease: Revisited.
DISCUSSION: In the 1980s and 1990s we used the Haemonetics Corporation's V-50 machine to perform intermittent whole blood (WB) exchange to treat severe complications of sickle cell disease (SCD.) The results were excellent, as previously described [1]. PMID: 31882437 [PubMed - in process]
Source: Annals of Clinical and Laboratory Science - October 31, 2019 Category: Laboratory Medicine Authors: Ballas SK Tags: Ann Clin Lab Sci Source Type: research

Hydroxyurea reduces cerebral metabolic stress in patients with sickle cell anemia
Chronic transfusion therapy (CTT) prevents stroke in selected patients with sickle cell anemia (SCA). We have shown that CTT mitigates signatures of cerebral metabolic stress, reflected by elevated oxygen extraction fraction (OEF), which likely drives stroke risk reduction. The region of highest OEF falls within the border zone, where cerebral blood flow (CBF) nadirs; OEF in this region was reduced after CTT. The neuroprotective efficacy of hydroxyurea (HU) remains unclear. To test our hypothesis that patients receiving HU therapy have lower cerebral metabolic stress compared with patients not receiving disease-modifying t...
Source: Blood - May 29, 2019 Category: Hematology Authors: Fields, M. E., Guilliams, K. P., Ragan, D., Binkley, M. M., Mirro, A., Fellah, S., Hulbert, M. L., Blinder, M., Eldeniz, C., Vo, K., Shimony, J. S., Chen, Y., McKinstry, R. C., An, H., Lee, J.-M., Ford, A. L. Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis Source Type: research

Sickle Cell Disease Subjects Have a Distinct Abnormal Autonomic Phenotype Characterized by Peripheral Vasoconstriction With Blunted Cardiac Response to Head-Up Tilt
Conclusion We have shown that SCD subjects are much more likely than non-SCD subjects to have impaired cardiac, but intact peripheral responses to orthostatic stress induced by HUT. These abnormal responses are associated with low baseline cardiac parasympathetic activity, independent of hemoglobin level. The classification of autonomic phenotypes based on HUT response may have potential use for predicting disease severity, guiding and targeting treatments/interventions to alleviate the risk of adverse outcomes in SCD. Ethics Statement All experiments were conducted at Children’s Hospital Los Angeles (CHLA). The ...
Source: Frontiers in Physiology - April 10, 2019 Category: Physiology 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