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 to iron overload and appropriate monitoring and treatment should be provided. Alloimmunization is also a common complication of transfusion and can lead to haemolytic transfusion reactions.
Source: ISBT Science Series - Category: Hematology Authors: Tags: Invited Review Source Type: research

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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 - Category: Hematology Source Type: research
Sickle cell disease (SCD) is one of the most common monogenic disorders worldwide and affects approximately 100,000 people in the United States alone. SCD can cause numerous complications, including anemia, pain, stroke, and organ failure, which can lead to death. Although there are a few disease-modifying treatments available to patients with SCD, the only current curative option is a hematopoietic stem cell transplant (HSCT). In this review, we will discuss the different approaches to allogeneic HSCT in the treatment of SCD and the outcomes of these approaches.
Source: Transfusion and Apheresis Science - Category: Hematology Authors: Source Type: research
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 - Category: Hematology Tags: Anemia Source Type: research
CONCLUSIONS: There is no evidence for managing adults, or children who do not have HbSS sickle cell disease. In children who are at higher risk of stroke and have not had previous long-term transfusions, there is moderate quality evidence that long-term red cell transfusions reduce the risk of stroke, and low quality evidence they also reduce the risk of other sickle cell disease-related complications. In primary and secondary prevention of stroke there is low quality evidence that switching to hydroxyurea with phlebotomy has little or no effect on the liver iron concentration. In secondary prevention of stroke there is lo...
Source: Cochrane Database of Systematic Reviews - Category: General Medicine Authors: Tags: Cochrane Database Syst Rev Source Type: research
Demystifying Medicine Lecture Series Although the fundamental molecular pathophysiology of sickle cell anemia was elucidated in studies of this genetic disease starting more than 70 years ago, it is only in about the last two decades that this knowledge has led to specific therapies. Indeed in the last decade serious attention has turned to the possibility of a genetic cure of this condition. Have we finally cured sickle cell anemia? Not quite, but we may be on the cusp. Really. In a dozen clinical trials planned or underway, some at the NIH Clinical Center, researchers are applying gene therapy to cure this well-studied g...
Source: Videocast - All Events - Category: General Medicine Tags: Upcoming Events Source Type: video
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
It’s only been seven years since scientists first learned how to precisely and reliably splice the human genome using a tool called CRISPR, making it possible to think about snipping out disease-causing mutations and actually cure, once and for all, genetic diseases ranging from sickle cell anemia to certain types of cancer and even blindness. Doctors are plunging ahead in search of ways to use the relatively new technology to start treating patients. In China last November, scientist Jiankui He stunned—and dismayed—the genetic community when he announced he had already used CRISPR, which many believe sti...
Source: TIME: Health - Category: Consumer Health News Authors: Tags: Uncategorized crispr Genetics Source Type: news
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
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