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Specialty: Hematology
Condition: Anemia

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Total 197 results found since Jan 2013.

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

Red cell distribution width is associated with future risk of incident stroke. The Tromsø Study.
Abstract Red cell distribution width (RDW), a measure of the variability in size of the circulating erythrocytes, is associated with cardiovascular morbidity and mortality. We aimed to investigate whether RDW was associated with incident stroke and case fatality in subjects recruited from the general population. Baseline characteristics were obtained from 25,992 subjects participating in the fourth survey of the Tromsø Study, conducted in 1994/95. Incident stroke was registered from inclusion until December 31, 2010. Cox regression models were used to calculate hazard ratios (HR) with 95 % confidence intervals ...
Source: Thrombosis and Haemostasis - August 20, 2015 Category: Hematology Authors: Lappegård J, Ellingsen TS, Skjelbakken T, Mathiesen EB, Njølstad I, Wilsgaard T, Brox J, Brækkan SK, Hansen JB Tags: Thromb Haemost 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

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

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

Two Sides of a Coin: Case Report of Unilateral Synangiosis and Contralateral Stroke Highlighting Consequences of Disease Progression and Efficacy of Revascularization in Sickle Cell Disease Associated Moyamoya Syndrome
We describe a challenging case where a patient with sickle cell disease undergoing standard of care management as prescribed by the Stroke Prevention Trial in Sickle Cell Anemia (STOP) and revascularization with pial synangiosis subsequently developed rapidly progressive disease in other cerebral vessels and suffered ischemic hemispheric stroke. This case demonstrates the success of management in accordance with American Heart Association (AHA) and American Stroke Association (ASA) guidelines, but also demonstrates critical areas where we lack understanding of disease progression.
Source: Acta Haematologica - December 8, 2021 Category: Hematology Source Type: research