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Specialty: Hematology
Condition: Pain
Education: Study

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Total 36 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

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

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

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

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

Alloimmunization in sickle cell disease: changing antibody specificities and association with chronic pain and decreased survival.
CONCLUSION: Alloimmunization in SCD may be associated with chronic pain, risk of end-organ damage, and shorter survival. These novel findings suggest new directions for the investigation of immune response-mediated pathways common to alloimmunization and chronic pain. PMID: 25444611 [PubMed - as supplied by publisher]
Source: Transfusion - December 1, 2014 Category: Hematology Authors: Telen MJ, Afenyi-Annan A, Garrett ME, Combs MR, Orringer EP, Ashley-Koch AE Tags: Transfusion Source Type: research

Blood transfusion and 30-day readmission rate in adult patients hospitalized with sickle cell disease crisis.
CONCLUSION: Our findings point to blood transfusion as a potential means to reduce the 30-day readmission rate among Medicaid patients hospitalized with sickle cell crisis. There is a need for a prospective study to examine the potential benefit and safety of simple blood transfusion for this purpose. PMID: 26126756 [PubMed - as supplied by publisher]
Source: Transfusion - June 30, 2015 Category: Hematology Authors: Nouraie M, Gordeuk VR Tags: Transfusion Source Type: research

What is the role of screening for pulmonary hypertension in adults and children with sickle cell disease?
Authors: Willen SM, Gladwin MT Abstract Patient case: An 18-year-old male patient with homozygous hemoglobin SS disease was evaluated for progressive dyspnea and elevated tricuspid regurgitant jet velocity (TRV) on echocardiography. The patient's case is described in detail in Lancet1 He had been treated with regular transfusions since childhood for stroke, had rare episodes of vaso-occlusive pain episodes, and did not take narcotic pain medications. He presented with progressive severe dyspnea on exertion and lower extremity edema. His laboratory tests were notable for a total hemoglobin level of 11.8 g/dL and hem...
Source: Hematology ASH Education Program - December 10, 2017 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Realizing Effectiveness Across Continents with Hydroxyurea: Enrollment and Baseline Characteristics of the Multicenter REACH Study in sub ‐Saharan Africa
This article is protected by copyright. All rights reserved.
Source: American Journal of Hematology - January 10, 2018 Category: Hematology Authors: Patrick T. McGann, Thomas N. Williams, Peter Olupot ‐Olupot, George A. Tomlinson, Adam Lane, José Luís Reis da Fonseca, Robert Kitenge, George Mochamah, Ham Wabwire, Susan Stuber, Thad A. Howard, Kathryn McElhinney, Banu Aygun, Teresa Latham, Brígida Tags: Research Article Source Type: research

Factor V Leiden G1691A and prothrombin G20210A mutations among Palestinian patients with sickle cell disease
ConclusionsFVL was more prevalent among SS patients compared to controls and it was associated with higher incidence of disease complications among SCD patients.
Source: BMC Hematology - January 16, 2018 Category: Hematology Source Type: research

Transfusion-transmitted malaria masquerading as sickle cell crisis with multisystem organ failure.
CONCLUSIONS: Clinical suspicion for transfusion-related adverse events, including hemolytic transfusion reactions and transfusion-transmitted infections, should be high in typically and atypically immunocompromised patient populations (like SCD), especially those on chronic transfusion protocols. Manual blood smear review aids in the evaluation of patients with SCD presenting with severe vaso-occlusive crisis and MSOF and can alert clinicians to the need for initiating aggressive therapy like RBC exchange and artesunate therapy. PMID: 29524230 [PubMed - as supplied by publisher]
Source: Transfusion - March 9, 2018 Category: Hematology Authors: Maier CL, Gross PJ, Dean CL, Chonat S, Ip A, McLemore M, El Rassi F, Stowell SR, Josephson CD, Fasano RM Tags: Transfusion Source Type: research