Curative treatment for severe sickle cell disease: allogeneic transplantation.

Curative treatment for severe sickle cell disease: allogeneic transplantation. Clin Adv Hematol Oncol. 2015 Apr;13(4):249-56 Authors: Oshrine B, Talano JA Abstract Sickle cell disease is an inherited hematologic disorder that in its severe form can result in substantial morbidity and early mortality. Patients with this disorder can suffer from severe pain, lung disease, and strokes, resulting in chronic debilitating conditions, end organ dysfunction, and organ failure. The health care costs of caring for these chronically ill patients are substantial. Allogeneic transplantation is a modality that has the potential to cure these patients. To date, matched sibling donor transplantation is widely accepted as a standard of care for pediatric patients. Utilizing alternative donors for transplant is still under investigation, as is transplant for adult patients with sickle cell disease. This review focuses on the most recent data for hematopoietic cell transplantation for patients with sickle cell disease. PMID: 26352583 [PubMed - in process]
Source: Clinical Advances in Hematology and Oncology - Category: Cancer & Oncology Tags: Clin Adv Hematol Oncol Source Type: research

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Abstract Sickle cell disease (SCD) is among the most common genetic diseases in the United States, affecting approximately 100,000 people. In the United States, SCD is characterized by a shortened life expectancy of only about 50 years in severe subtypes, significant quality-of-life impairments, and increased healthcare utilization and spending. SCD is characterized by chronic hemolytic anemia, vaso-occlusion, and progressive vascular injury affecting multiple organ systems. The pathophysiology is directly related to polymerization of deoxygenated hemoglobin, leading to a cascade of pathologic events including ery...
Source: The American Journal of Managed Care - Category: Health Management Authors: Tags: Am J Manag Care Source Type: research
Inherited hemoglobin disorders, including beta-thalassemia (BT) and sickle-cell disease (SCD) are the most common monogenic diseases worldwide, with a global carrier frequency of over 5%. With migration they are becoming more common worldwide, making their management and care an increasing concern for health care systems.BT is characterized by an imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and compensatory haemopoietic expansion. Globally, there are over 25,000 births each year with transfusion-dependent thalassemia (TDT). The current available tr...
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Source Type: research
Purpose of review Red cell transfusions are one of the most common and important therapies used for patients with sickle cell disease (SCD). For prevention of strokes, there is abundant evidence that transfusions are efficacious, whereas for other indications, such as prevention of pain, there are less data. Nonetheless, with few therapeutic options, the use of transfusion for prevention of acute pain has increased in children and adults with SCD without a clear understanding of its benefits. Recent findings Although it makes conceptual sense that red cell transfusions would prevent pain that arises from vaso-occlusio...
Source: Current Opinion in Hematology - Category: Hematology Tags: TRANSFUSION MEDICINE AND IMMUNOHEMATOLOGY: Edited by Steven L. Spitalnik Source Type: research
Sickle cell disease (SCD) is a medical condition in which chronic pain is common and negatively impacts psychosocial function and quality of life. Although the brain mechanisms underlying chronic pain are well studied in other painful conditions, the brain mechanisms underlying chronic pain and the associated psychosocial comorbidities are not well established in SCD. A growing literature demonstrates the effect of treatment of chronic pain, including pharmacological and nonpharmacological treatments, on brain function. The present systematic review aimed to (1) determine the effects of chronic pain and psychosocial comorb...
Source: Pain - Category: Anesthesiology Tags: Systematic Reviews and Meta-Analyses Source Type: research
Sickle cell disease (SCD) is a severe chronic disease that leads to premature mortality caused by serious complications of the disease such as acute chest syndrome, stroke, and sepsis. Patients presenting to the emergency department (ED) with pain due to vaso-occlusive crisis (VOC) are at a higher risk for complications, making it imperative that emergency nurses, nurse practitioners, and physicians are knowledgeable about SCD and understand the other associated complications besides VOC. Because of the complexity of disease and misperceptions about SCD among ED nurses, physicians, and nurse practitioners, a quality improv...
Source: Advanced Emergency Nursing Journal - Category: Emergency Medicine Tags: PROCEDURAL COLUMN Source Type: research
Researchers at Florida Atlantic University have developed a microfluidic chip that can rapidly assess blood samples from sickle cell disease patients to help monitor the disease. The technique is much faster and more convenient than traditional optic...
Source: Medgadget - Category: Medical Devices Authors: Tags: Diagnostics Medicine Source Type: blogs
CONCLUSIONS: Patients exhibiting greater daily HbA decrement were more likely to have multiple emergency department visits or admissions for sickling crises. Modulating HbA decrement may merit study as an intermediate metric for interventions to improve outcomes in hemoglobin SS disease. PMID: 31161685 [PubMed - as supplied by publisher]
Source: Transfusion - Category: Hematology Authors: Tags: Transfusion Source Type: research
In this study, we used HUT as the means to provide an all-encompassing assessment of cardiac and/or peripheral autonomic function in normal controls, SCD subjects and non-SCD subjects with chronic anemia. We hypothesized that by identifying different categories of HUT response among these subjects, we would be able to isolate the autonomic phenotypes that might place SCD subjects at increased risk for microvascular occlusion and VOC. We then employed the causal modeling approach, which utilizes signal analysis and system identification techniques, to probe and disentangle the functional mechanisms involved in the cardiovas...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
In this study, we reviewed the outcome of SCD patients who underwent transplant at our institution using standard protocols (NMA regimen in patients ≥14 years and myeloablative regimen in
Source: Blood - Category: Hematology Authors: Tags: 732. Clinical Allogeneic Transplantation: Results: Poster II Source Type: research
This study examined influenza vaccination in patients with SCD as a potential marker of quality of care delivery. The study population included black individuals aged 1 to =1 diagnosis of stroke, TIA or epilepsy/recurrent seizures); cardio pulmonary complications (>=1 diagnosis of chronic pulmonary heart disease, pulmonary hypertension, malaise and fatigue, edema, chest pain or hypoxemia); kidney disease (>=1 diagnosis of chronic kidney disease/renal failure, proteinuria), avascular necrosis or ulcer of lower limbs (adapted from Afenyi-Annan, 2008, Candrilli. 2011, Elmariah. 2014). A total of 1544 patients with SCD w...
Source: Blood - Category: Hematology Authors: Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster II Source Type: research
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