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Specialty: Hematology
Condition: Sickle Cell Anemia

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Total 508 results found since Jan 2013.

Elevated Levels of CD64 MFI on Monocyte Subsets Are Associated with a History of Stroke in Sickle Cell Disease
Conclusion: Levels of monocyte subsets have been shown to correlate with clinical outcomes in non-SCD stroke, but to our knowledge this is the first study to examine their roles in ischemic stroke in SCD. The pathophysiology of stroke in SCD is unique and the role of monocytes in it deserves separate study from the role of monocytes in non-SCD stroke. We saw that while monocyte subsets were associated with a history of ischemic stroke, CD64 MFI on all monocyte subsets showed a strong association. We wonder if this may be due to CD64 MFI being a marker for cells which are more adherent to endothelium as has been shown in pr...
Source: Blood - November 21, 2018 Category: Hematology Authors: Curtis, S. A., Balbuena-Merle, R., Devine, L., Zelterman, D., Roberts, J. D., Dearborn-Tomazos, J., Sansing, L., Hendrickson, J. E. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster I Source Type: research

Outcome of overt stroke in sickle cell anaemia, a single institution's experience
Summary Stroke is a traumatic complication in sickle cell anaemia (SCA) that is associated with significant morbidity and a risk of recurrent overt stroke of 2·2–6·4 events per 100 patient‐years. A retrospective study was performed on all paediatric SCA patients diagnosed with a history of overt stroke between 1997 and 2010. A total of 31 children with SCA had new onset overt stroke. The mean age of the active patients (n = 27) was 17·9 years (range 6·8–27·6 years) with a total period of observation of 305 patient‐years. Twenty‐two of 27 (81%) were receiving long term red blood cell transfusions and 16 (...
Source: British Journal of Haematology - February 27, 2014 Category: Hematology Authors: Suvankar Majumdar, Morgan Miller, Majid Khan, Catherine Gordon, Amy Forsythe, Mary G. Smith, Gail Megason, Rathi Iyer Tags: Research Paper Source Type: research

Practice Patterns in the Use of MRI/MRA and Chronic Transfusion Therapy for Monitoring and Treatment of Stroke in Pediatric Patients with Sickle Cell Anemia
Conclusions: The results of the DISPLACE practice patterns survey suggested nearly universal adoption of CRCT for children with abnormal TCD and prior overt stroke across sites. The use of MRI/MRA in detecting potential cerebrovascular abnormalities in the absence of clinical neurologic symptoms and for monitoring patients on CRCT was much more variable, which likely reflects the minimal amount of guidance for the use of MRI/MRA in SCA. Notably, a number of sites were using MRI/MRA to screen pediatric patients for cerebrovascular abnormalities and to monitor patients on CRCT. The variability in responses for MRI/MRA highli...
Source: Blood - November 21, 2018 Category: Hematology Authors: Schlenz, A. M., Mueller, M., Phillips, S., Melvin, C., Adams, R. J., Kanter, J. Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III Source Type: research

Unusually High Prevalence of Stroke and Cerebral Vasculopathy in Hemoglobin SC Disease: A Retrospective Single Institution Study
Conclusion: There is a distinct subset of individuals with HbSC who developed overt, silent stroke, and cerebral vasculopathy. A female predominance and association with Rec VOCs were identified in our cohort; however, larger clinical trials are needed to identify and confirm specific clinical and laboratory markers associated with stroke and vasculopathy in HbSC disease.Acta Haematol
Source: Acta Haematologica - November 8, 2021 Category: Hematology Source Type: research

Interleukin-1 receptor inhibition reduces stroke size in a murine model of sickle cell disease.
Abstract Sickle cell disease (SCD) is associated with chronic hemolytic anemia and a heightened inflammatory state. The causal role of inflammatory pathways in stroke associated with SCD is unclear. Therefore, the hypothesis that deletion of the non-hematopoietic interleukin-1 receptor (IL-1R) pool may be beneficial in SCD was pursued. Since potential deleterious effects of IL-1R signaling in SCD could be mediated via downstream production of interleukin-6 (IL-6), the role of the non-hematopoietic IL-6 pool was also addressed. Bone marrow transplantation (BMT) from SCD to wild-type (WT) recipient mice was used to ...
Source: Haematologica - August 12, 2020 Category: Hematology Authors: Venugopal J, Wang J, Mawri J, Guo C, Eitzman D Tags: Haematologica Source Type: research

β(S) globin gene haplotype and the stroke risk among Egyptian children with sickle cell disease.
CONCLUSION: This study provides a relevant contribution to our understanding of the anthropological and historical background of the population in Egypt where Benin haplotype is the commonest β(S) globin gene haplotype and homozygous Benin/Benin is associated with higher stroke risk than other haplotypes. PMID: 29157167 [PubMed - as supplied by publisher]
Source: Hematology - November 23, 2017 Category: Hematology Tags: Hematology Source Type: research

A Genetic Variant in GOLGB1 Affects Golgi Function and Stroke Risk in Patients with Sickle Cell Disease
Conclusions: Our study has shown that a coding variant in GOLGB1, identified as protective against risk of stroke in patients with SCD, has significant effects on Golgi function in SCD samples. We observed that having the GOLGB1 Y1212C variant resulted in more compact and less fragmented Golgi apparatus. Proteomic analysis showed that SCD patients with the GOLGB1 variant also had significantly lower levels of proteins involved in platelet activation and Golgi trafficking. Our findings suggest a novel role for the Golgi apparatus in controlling protein flux that modulates risk of stroke in SCD.DisclosuresNo relevant conflic...
Source: Blood - November 21, 2018 Category: Hematology Authors: Kotu, V., Dubrelle, J., Baker, J., Jenson, K., Flanagan, J. M. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster III Source Type: research

Genetic mapping and exome sequencing identify 2 mutations associated with stroke protection in pediatric patients with sickle cell anemia
Stroke is a devastating complication of sickle cell anemia (SCA), occurring in 11% of patients before age 20 years. Previous studies of sibling pairs have demonstrated a genetic component to the development of cerebrovascular disease in SCA, but few candidate genetic modifiers have been validated as having a substantial effect on stroke risk. We performed an unbiased whole-genome search for genetic modifiers of stroke risk in SCA. Genome-wide association studies were performed using genotype data from single-nucleotide polymorphism arrays, whereas a pooled DNA approach was used to perform whole-exome sequencing. In combina...
Source: Blood - April 18, 2013 Category: Hematology Authors: Flanagan, J. M., Sheehan, V., Linder, H., Howard, T. A., Wang, Y.-D., Hoppe, C. C., Aygun, B., Adams, R. J., Neale, G. A., Ware, R. E. Tags: Pediatric Hematology, Red Cells, Iron, and Erythropoiesis Source Type: research

The Role of Hydroxyurea to Prevent Silent Stroke in Sickle Cell Disease: Systematic Review and Meta-Analysis
Chronic blood transfusions are standard of care for stroke prevention in sickle cell disease. We evaluated hydroxyurea's efficacy in preventing silent stroke. We searched for randomized controlled trials (RCTs) and observational studies on Pubmed, CENTRAL, Embase, and Web of Science without using language/time limits. Eligible studies compared hydroxyurea with transfusions or observation to prevent silent stroke. This systematic review adheres to the Cochrane guidelines. Data were pooled using random effects model using STATA to perform meta-analysis. Methodological quality of RCTs was investigated using the Cochrane risk ...
Source: Blood - November 21, 2018 Category: Hematology Authors: Hasson, C., Veling, L., Rico, J. F., Mhaskar, R. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research

High mortality among children with sickle cell anemia and overt stroke who discontinue blood transfusion after transition to an adult program.
CONCLUSION: Efforts must be made to maintain adequate chronic simple or exchange blood transfusion for children with SS and stroke after transition to adult care. PMID: 26593779 [PubMed - as supplied by publisher]
Source: Transfusion - November 23, 2015 Category: Hematology Authors: McLaughlin JF, Ballas SK Tags: Transfusion Source Type: research

Frequent red cell transfusions reduced vascular endothelial activation and thrombogenicity in children with sickle cell anemia and high stroke risk
Abstract Stroke is one of the most disabling complications of sickle cell anemia (SCA). The molecular mechanisms leading to stroke in SCA or by which packed red blood cell (PRBC) transfusion prevents strokes are not understood. We investigated the effects of PRBC transfusion on serum biomarkers in children with SCA who were at high‐risk for stroke. Serum samples from 80 subjects were analyzed, including baseline, study exit time point and 1 year after study exit. Forty of the 80 samples were from subjects randomized to standard care and 40 from transfusion arm. Samples were assayed for levels of BDNF, sVCAM‐1, sICAM‐...
Source: American Journal of Hematology - August 31, 2013 Category: Hematology Authors: Hyacinth I. Hyacinth, Robert J. Adams, Jenifer H. Voeks, Jacqueline M. Hibbert, Beatrice E. Gee Tags: Research Article Source Type: research

Middle Cerebral Artery Velocities Are Inversely Related to Hemoglobin Levels and Acutely Drop in Response to RBC Transfusion: Implications for Stroke Screening in SCD
DiscussionThese data show a striking relationship between anemia and TCD velocity, and that RBC transfusion can alter MCA velocity. Even an 11% increase in hemoglobin such as that seen in the low-responder in Figure 3 could change a patient classification from high-risk to conditional TCD. These data also show the significant sensitivity of MCA velocity to the depth of measurement (Figure 2 & 3). The investigators in the STOP study went to extreme ends to standardize these measurements. Nonetheless, TCD measures may not be done with such rigor in clinical settings. It is clear that standard TCD screening has been of tr...
Source: Blood - November 21, 2018 Category: Hematology Authors: Perumbeti, A., Carreras, O., Detterich, J. A., Shah, P., Sunwoo, J., Khoo, M., Wood, J. C., Coates, T. D. Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research

Genetic modulators of fetal hemoglobin expression and ischemic stroke occurrence in African descendant children with sickle cell anemia
This study aimed to investigate the influence of genetic variants on the levels of fetal hemoglobin (Hb F) and biochemical parameters related with chronic hemolysis, as well as on ischemic stroke risk, in ninety-one unrelated SCA patients, children of sub-Saharan progenitors. Our results show that a higher Hb F level has an inverse relationship with the occurrence of stroke, since the group of patients who suffered stroke presents a significantly lower mean Hb F level (5.34  ± 4.57% versus 9.36 ± 6.48%;p = 0.024). Furthermore, the co-inheritance of alpha-thalassemia improves the chronic hemolytic pattern, evid...
Source: Annals of Hematology - September 1, 2019 Category: Hematology Source Type: research