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Hematopoietic Stem Cell Transplantation for Sickle Cell Disease: State of the Science
This article is protected by copyright. All rights reserved.
Source: European Journal of Haematology - September 1, 2014 Category: Hematology Authors: Julie‐An Talano, Mitchell S. Cairo Tags: Review Article 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

Smoothing the crescent curve: sickle cell disease.
Authors: Talano JA, Cairo MS Abstract Sickle cell disease (SCD) is an inherited disorder secondary to a point mutation at the sixth position of the beta chain of human hemoglobin that results in the replacement of valine for glutamic acid. This recessive genetic abnormality precipitates the polymerization of the deoxygenated form of hemoglobin S that induces a major distortion of red blood cells (sickle red blood cells), which decreases sickle red blood cell deformability, leading to chronic hemolysis and vasoocclusion. These processes can result in severe complications, including chronic pain, end organ dysfunctio...
Source: Hematology ASH Education Program - February 20, 2015 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program 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

Sickle cell disease: when and how to transfuse.
Authors: Howard J Abstract Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome,...
Source: Hematology ASH Education Program - December 4, 2016 Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research

Hemopericardium under dabigatran for stroke prevention in atrial fibrillation
We report a polymorbid 75-year old male under a therapy with dabigatran, valsartan, amlodipine, nicorandil, furosemide, atorvastatin, bisoprolol, metformin, tizanidine, pantoprazole, and tramadol. He suffered from chest pain for 4 months. Coronary angiography showed only ectatic coronary arteries. He started taking nonsteroidal anti-inflammatory drugs. He was hospitalized because of dyspnea starting 10 days before admission, melena, and renal failure. Hemopericardium was diagnosed and pericardiocentesis yielded 2000 ml hemorrhagic fluid. Review of previous echocardiograms showed a 4 mm echo-free space, epicardial fat o...
Source: Blood Coagulation and Fibrinolysis - February 11, 2017 Category: Hematology Tags: Case Reports Source Type: research

Outcomes of Unrelated Donor Stem Cell Transplant with Post-Transplant Cyclophosphamide for Graft-versus-Host Disease Prophylaxis in Patients with Severe Sickle Cell Disease
Sickle cell disease (SCD) is a hemoglobinopathy affecting 1 in every 500 African American newborns and an estimated 100,000 persons in the United States [1]. Vascular occlusion caused by the sickled RBCs leads to decreased perfusion in target organs and manifests clinically as vaso-occlusive pain crisis, stroke (cerebral infarction), pulmonary restrictive disease, and pulmonary hypertension [2]. Despite advances in supportive care, including the use of hydroxyurea (HU), RBC transfusion, and iron chelation therapy, patients with SCD continue to experience considerable morbidity and mortality in their lifetime.
Source: Biology of Blood and Marrow Transplantation - October 20, 2017 Category: Hematology Authors: Hemalatha G. Rangarajan, Rolla Abu-Arja, Vinita Pai, Gregory M.T. Guilcher, Sandeep Soni Source Type: research

Outcomes of Unrelated Donor Stem Cell Transplantion with Post-Transplant Cyclophosphamide for Graft-versus-Host Disease Prophylaxis in Patients with Severe Sickle Cell Disease
Sickle cell disease (SCD) is a hemoglobinopathy affecting 1 in every 500 African American newborns and an estimated 100,000 persons in the United States [1]. Vascular occlusion caused by the sickled RBCs leads to decreased perfusion in target organs and manifests clinically as vaso-occlusive pain crisis, stroke (cerebral infarction), pulmonary restrictive disease, and pulmonary hypertension [2]. Despite advances in supportive care, including the use of hydroxyurea (HU), RBC transfusion, and iron chelation therapy, patients with SCD continue to experience considerable morbidity and mortality in their lifetime.
Source: Biology of Blood and Marrow Transplantation - October 20, 2017 Category: Hematology Authors: Hemalatha G. Rangarajan, Rolla Abu-Arja, Vinita Pai, Gregory M.T. Guilcher, Sandeep Soni 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

Simple chronic transfusion therapy, a crucial therapeutic option for sickle cell disease, improves but does not normalize blood rheology: What should be our goals for transfusion therapy?
Abstract Sickle cell anemia is characterized by a mutation resulting in the formation of an abnormal beta-hemoglobin called hemoglobin S. Hemoglobin S polymerizes upon deoxygenation, causing impaired red blood cell deformability and increased blood viscosity at equivalent hematocrits. Thus, sickle cell disease is a hemorheologic disease that results in various pathologic processes involving multiple organ systems including the lungs, heart, kidneys and brain. Red blood cell mechanics and the perturbations on blood flow-endothelial interaction underlie much of the pathology found in sickle cell disease. Transfusion...
Source: Clinical Hemorheology and Microcirculation - April 5, 2018 Category: Hematology Authors: Detterich JA Tags: Clin Hemorheol Microcirc Source Type: research

Genetic modifiers of severity in sickle cell disease.
Abstract Sickle cell disease (SCD) is one of the most common single disease disorders world-wide. It is remarkable for its clinical heterogeneity, even among individuals with identical genotypes. Some individuals experience morbidity and mortality in early childhood, while others have a relatively mild course, and normal or near normal life expectancy. Many clinical complications are associated with SCD; most notably frequent pain episodes, stroke, acute chest syndrome, avascular necrosis, nephropathy, retinopathy and pulmonary hypertension. While the effects of higher fetal hemoglobin (HbF) levels, UGTA1A polymor...
Source: Clinical Hemorheology and Microcirculation - April 5, 2018 Category: Hematology Authors: Chang AK, Ginter Summarell CC, Birdie PT, Sheehan VA Tags: Clin Hemorheol Microcirc Source Type: research

Biomarker signatures of sickle cell disease severity.
Abstract Identifying sickle cell disease patients at high risk of complications could lead to personalized treatment and better prognosis but despite many advances prediction of the clinical course of these patients remains elusive. We propose a system-type approach to discover profiles of multiple, common biomarkers that correlate with morbidity and mortality in sickle cell disease. We used cluster analysis to discover 17 signatures of 17 common circulating biomarkers in 2320 participants of the Cooperative Study of Sickle Cell Disease, and evaluated the association of these signatures with risk for stroke, pain,...
Source: Blood Cells, Molecules and Diseases - May 16, 2018 Category: Hematology Authors: Du M, Van Ness S, Gordeuk V, Nouraie SM, Nekhai S, Gladwin M, Steinberg MH, Sebastiani P Tags: Blood Cells Mol Dis Source Type: research

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