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Specialty: Hematology
Condition: Pain

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Total 53 results found since Jan 2013.

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

Pain and other non‐neurological adverse events in children with sickle cell anemia and previous stroke who received hydroxyurea and phlebotomy or chronic transfusions and chelation: Results from the SWiTCH clinical trial
Conclusions: In children with SCA and prior stroke, monthly transfusions and daily iron chelation provided superior protection against acute vaso‐occlusive pain SAEs when compared to hydroxyurea and monthly phlebotomy.
Source: American Journal of Hematology - July 16, 2013 Category: Hematology Authors: Ofelia Alvarez, Nancy A. Yovetich, J. Paul Scott, William Owen, Scott T. Miller, William Schultz, Alexandre Lockhart, Banu Aygun, Jonathan Flanagan, Melanie Bonner, Brigitta U. Mueller, Russell E. Ware, Tags: Research Article Source Type: research

Stroke and presence of patent foramen ovale in sickle cell disease
AbstractSickle cell disease (SCD) is an inherited monogenic hemoglobinopathy characterized by formation of sickle erythrocytes under conditions of deoxygenation. Sickle erythrocytes can lead to thrombus formation and vaso-occlusive episodes that may result in hemolytic anemia, pain crisis and multiple organ damage. Moreover, SCD is characterized by endothelial damage, increased inflammatory response, platelet activation and aggravation, and activation of both the intrinsic and the extrinsic coagulation pathways. Cerebrovascular events constitute an important clinical complication of SCD. Children with SCD have a 300-fold h...
Source: Journal of Thrombosis and Thrombolysis - February 26, 2021 Category: Hematology Source Type: research

Thrombocytopenia with acute ischemic stroke and bleeding in a patient newly vaccinated with an adenoviral vector-based COVID-19 vaccine
We describe the first Danish case of presumed inflammatory and thrombotic response to vaccination with an adenoviral (ChAdOx1) vector based COVID-19 vaccine (AZD1222). The case describes a 60-year old woman, who was admitted with intractable abdominal pain seven days after receiving the vaccine. Computer Tomography (CT) of the abdomen revealed bilateral adrenal hemorrhages. On the following day she developed a massive right sided ischemic stroke and Magnetic Resonance Imaging (MRI) angiography showed occlusion of the right internal carotid artery. The ischemic area was deemed too large to offer reperfusion therapy. During ...
Source: Thrombosis and Haemostasis - April 20, 2021 Category: Hematology Authors: Rolf Ankerlund Blauenfeldt S øren Risom Kristensen Siw Leiknes Ernstsen Claudia Christina Hilt Kristensen Claus Ziegler Simonsen Anne-Mette Hvas Source Type: research

Mast Cells Contribute to Brain Microvascular Permeability in Sickle Cell Disease
We examined the ability of mast cells to stimulate P-selectin expression and BBB permeability via ER stress in a sickle microenvironment.We isolated MCs from HbAA-BERK and HbSS-BERK, control and sickle mice, respectively; incubated them in vitro and collected mast cell conditioned media (MCCM) from HbAA MCs and HbSS MCs. Normal mouse brain microvascular endothelial cells (mBMECs) were treated with unconditioned MCCM, HbAA MCCM, or HbSS MCCM to examine the effect of mast cell activation on endothelium. We observed increased mast cell activity in HbSS mice evinced by significantlyhigher plasma and skin histamine levels, comp...
Source: Blood - November 21, 2018 Category: Hematology Authors: Mittal, A. M., Tran, H., Sagi, V., Nguyen, A., Luk, K., Nguyen, J., Lei, J., Gupta, K. Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Sickle Cell Disease-Role of Coagulation and Inflammation in Pathophysiology Source Type: research

Transfusion Therapy in a Multi-Ethnic Sickle Cell Population Real-World Practice. a Preliminary Data Analysis of Multicentre Survey
Conclusion. The transfusional approach is similar in HbSS, b°-thal/HbS and b+-thal/HbS patients with similar indications, prevalently VOCs and anemia. The significant higher age in Caucasian cohort and the consequent long term follow up could be the cause of variable therapeutic approach observed, however Hydroxycarbamide seemed to be the therapy more frequently used and finally suggested to manage chronic manifestations.Figure.DisclosuresOriga: Apopharma: Honoraria; Novartis: Honoraria; Bluebird Bio: Consultancy; Cerus Corporation: Research Funding. Forni: Apopharma: Other: DSM Board; Celgene: Research Funding; Novart...
Source: Blood - November 21, 2018 Category: Hematology Authors: Graziadei, G., Sainati, L., Bonomo, P., Venturelli, D., Masera, N., Casale, M., Vassanelli, A., Lodi, G., Piel, F. B., Voi, V., De Franceschi, L., Rigano, P., Quota, A., Notarangelo, L. D., Russo, G., Rosso, R., Allo, M., D'Ascola, D., Facchini, E., Macch Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research

Transfusion therapy for sickle cell disease
The clinical presentations of sickle cell disease include acute pain, acute chest syndrome, stroke and acute anaemia and blood transfusion therapy can be used effectively in the emergency setting for the treatment of many of these complications. Long‐term blood transfusion therapy is also used for the prevention of disease complications with most evidence for its use in primary stroke prevention. Transfusion can be given as a simple or top‐up transfusion or as an exchange transfusion and donor red cells should be haemoglobin S negative and as a minimum matched for ABO, full Rh and Kell type. Repeated transfusions lead ...
Source: ISBT Science Series - January 29, 2016 Category: Hematology Authors: J. Howard, S. E. Robinson Tags: Invited Review Source Type: research

Characterization and treatment of congenital thrombotic thrombocytopenic purpura
In conclusion, prespacer mutations are associated with earlier development of cTTP symptoms. Prophylactic ADAMTS13 replacement decreases the risk of end-organ damage such as ischemic stroke and resolved previously unrecognized symptoms in patients with nonovert disease.
Source: Blood - April 10, 2019 Category: Hematology Authors: Alwan, F., Vendramin, C., Liesner, R., Clark, A., Lester, W., Dutt, T., Thomas, W., Gooding, R., Biss, T., Watson, H. G., Cooper, N., Rayment, R., Cranfield, T., van Veen, J. J., Hill, Q. A., Davis, S., Motwani, J., Bhatnagar, N., Priddee, N., David, M., Tags: Thrombocytopenia, Platelets and Thrombopoiesis, Clinical Trials and Observations Source Type: research

Blood Transfusion Frequency and Indications in Yemeni Children with Sickle Cell Disease.
Conclusion: Intermittent blood transfusion remains a common practice for the management of children with acute SCD complications. Main indications were acute anemic crises, severe pain crises, ACS, and stroke. In limited resource settings, such as Yemen, conservative transfusion policy appears to be appropriate. PMID: 32908695 [PubMed]
Source: Anemia - September 13, 2020 Category: Hematology Tags: Anemia Source Type: research

Correlates of ADL difficulty in a large hemodialysis cohort
Abstract Needing assistance with activities of daily living (ADL) is an early indicator of functional decline and has important implications for individuals' quality of life. However, correlates of need for ADL assistance have received limited attention among patients undergoing maintenance hemodialysis (HD). A multicenter cohort of 742 prevalent HD patients was assessed in 2009–2011 and classified as frail, prefrail and nonfrail by the Fried frailty index (recent unintentional weight loss, reported exhaustion, low grip strength, slow walk speed, low physical activity). Patients reported need for assistance with 4 ADL ta...
Source: Hemodialysis International - October 9, 2013 Category: Hematology Authors: Nancy G. Kutner, Rebecca Zhang, Richard M. Allman, C. Barrett Bowling Tags: Original Article Source Type: research

Markers of endothelial dysfunction differ between subphenotypes in children with sickle cell disease
Abstract: In adult patients with sickle cell disease two distinct subphenotypes have previously been defined: patients with the viscosity-vaso-occlusion subphenotype (VVO) suffer mainly from vaso-occlusive pain crises and have a relatively high hemoglobin concentration. Patients classified as the hemolysis-endothelial dysfunction subphenotype (HED) suffer from stroke and pulmonary hypertension and have an elevated concentration of lactate dehydrogenase. However, this classification is not possible in children due to low rates of complications. We used laboratory markers to classify children into the two subphenotypes, and ...
Source: Thrombosis Research - October 31, 2013 Category: Hematology Authors: Veronica van der Land, Marjolein Peters, Bart J. Biemond, Harriët Heijboer, Cornelis L. Harteveld, Karin Fijnvandraat Tags: Platelets and Cell Biology Source Type: research

Factors Associated with Survival in a Contemporary Adult Sickle Cell Disease Cohort
We examined the relationship of clinical differences among sickle cell disease (SCD) patients in order to understand the major contributors to early mortality in a contemporary cohort. Survival data were obtained for 542 adult subjects who were enrolled since 2002 at three university hospitals in the southeast United States. Subjects were followed for a median of 9.3 years. At enrollment, clinical parameters were collected, including hemoglobin (Hb) genotype, baseline laboratory values, comorbidities, and medication usage. Levels of soluble adhesion molecules were measured for a subset of 87 subjects. The relationship of c...
Source: American Journal of Hematology - January 30, 2014 Category: Hematology Authors: Hany Elmariah, Melanie E. Garrett, Laura M. Castro, Jude Jonassaint, Kenneth I. Ataga, James Eckman, Allison E. Ashley‐Koch, Marilyn J. Telen Tags: Research Article Source Type: research

Implications of glycoprotein VI for theranostics.
Abstract Glycoprotein VI (GPVI), a membrane glycoprotein solely expressed in platelets and megakaryocytes, plays a critical role in thrombus formation due to collagen/GPVI-mediated platelet activation and adhesion. Recent studies have shown that surface expression of GPVI on circulating platelets is enhanced in acute cardiovascular diseases such as myocardial infarction and ischaemic stroke. Increased GPVI levels are associated with poor clinical outcome and are an early indicator for imminent myocardial infarction in patients with chest pain. The soluble form of the dimeric GPVI fusion protein (sGPVI-Fc) binds wi...
Source: Thrombosis and Haemostasis - February 20, 2014 Category: Hematology Authors: Gawaz M, Vogel S, Pfannenberg C, Pichler B, Langer H, Bigalke B Tags: Thromb Haemost Source Type: research