Sickle Cell Disease - Crossref

American Society of Hematology 2021 guidelines for sickle cell disease: stem cell transplantation

"Abstract Background: Sickle cell disease is a life-limiting inherited hemoglobinopathy with severe complications and impacts quality of life. " The only curative treatment for SCD is Hematopoietic stem cell transplantation, according to data, but guidelines are still needed to explain how to use HSCT in clinical research. The Mayo Evidence-Based Practice Research Program supported the guideline design process, which included systematic evidence reviews. The panel accepted eight recommendations to help patients and providers assess how people with SCD should plan about the timing and type of HSCT. Conclusions: No randomized controlled clinical trials for HSCT in SCD were found in the study, so all recommendations are based on very little confidence in the evidence. The key recommendations include including HSCT for people with neurologic disease or recurrent acute chest syndrome at an early age, as well as improved nonmyeloablative regimens.

Source link: https://doi.org/10.1182/bloodadvances.2021004394c

Clinical correlation of positive direct antiglobulin tests in patients with sickle cell disease

"Abstract" says Robert Abbott Theopathic anemia is a disorder that affects patients with sickle cell disease and can be extremely relevant. The antigenic stimulation of chronic red blood cell transfusions, increased autoantibody production, RBC membrane defects, and functional asplenism are all typical of immune-mediated hemolysis, which may influence the onset and severity of immune-mediated hemolysis. On the surface, the patient's RBCs had IgG and complement, while others of the other patient had IgG and complement, while others of the other patient had IgG without complement. ".

Source link: https://doi.org/10.21307/immunohematology-2019-975

Directed blood donor program decreases donor exposure for children with sickle cell disease requiring chronic transfusion

"Further, the use of extended red blood cell antigen matching for C, E, and K has been well documented in a clinical trial context but not so well investigated in a typical care setting. " The aim of this research is to determine the success of reducing alloimmunization matching for C, E, and K, as well as the extent of donor sensitivity in a targeted blood donor campaign. In a cohort of children with SCD who received regular donor blood transfusions, the rate of alloimmunization and reduction of donor exposure were determined over the course of one year. Extended RBC antigen matching has the same benefit as in a clinical trial setting for patients with SCD receiving blood transfusion therapy.

Source link: https://doi.org/10.21307/immunohematology-2019-141

Hyperhemolytic transfusion reaction attributable to anti-Fy3 in a patient with sickle cell disease

"Abstract" is a sentence in which hyperhemolytic transfusion reaction attributed to anti-Fy3 in a 30-year-old African woman with a history of sickle cell disease is documented. The patient was admitted to a vaso-occlusive sickle cell disease, and received four units of packed RBCs secondary to worsening symptomatic anemia. The patient's antibody test and identification confirmed anti-V and anti-E, as well as anti-M, -B, -V, -Sl, a, and a cold agglutinin with potential anti-I specificity on admission. Transfused RBC units that lacked E,C,Jk b,N,V,and S were transfused. The patient's RBCs were found with IgG, according to the DAT. A delayed hemolytic transfusion reaction due to anti-Fy3 was suspected, and IVIG and high-dose prednisone were able to treat patients with delayed hemolytic transfusion reaction secondary to anti-Fy3 and hyperhemolysis secondary to anti-Fy3. This is the first reported case of hyperhemolysis in sickle cell disease attributable to anti-Fy3 antibodies, to the best of our knowledge.

Source link: https://doi.org/10.21307/immunohematology-2019-263

Transfusion of multiple units of Js(b+) red blood cells in the presence of anti-Js b in a patient with sickle β-thalassemia disease and a review of the literature

"A monocyte monolayer assay was applied to the patient's blood and plasma samples obtained 2 and 9 days after transfusion of the Js RBCs to determine the potential clinical significance of the anti-Js b. " After transfusion, the patient's anti-Js b's reactivity soared from 2. 3 percent on day 2 to a high degree of 66. 5 percent one week later. Following the transfusions, however, increased RBC survival was predicted by the relatively fast decline of the HbA 1 fraction. Anti-Js b may have little risk of causing overt hemolysis, according to the present case and others in the literature. However, even modestly elevated RBC destruction in patients with underlying hematologic disease may cause problems clinically, and so transfusion of Js RBCs should be avoided. ".

Source link: https://doi.org/10.21307/immunohematology-2019-324

In search of red blood cells for alloimmunized patients with sickle cell disease

"Abstract Patients with sickle cell disease generally require transfusions with RBC components, which exposes them to a variety of, possibly foreign antigens, possibly prompting them to produce an antibody or antibodies to the antigens they lack. Once a clinically relevant antibody is isolated, RBCs lacking the appropriate antigen should be transplanted. Often patients with SCD have multiple antibodies in their blood transfusion service, prompting the search for compatible RBCs. About 33% of the RBC's requests for RBC services between January 2005 and June 2006 were for alloimmunized patients with SCD. SCD-imunized patients with SCD's use and efficiency in this paper will discuss the use and success of the ARDP and testing laboratories attached to the National Reference Laboratory for Blood Group Serology at the American Red Cross in finding compatible RBCs for alloimmunized patients with SCD. ".

Source link: https://doi.org/10.21307/immunohematology-2019-371

Transfusion practices for patients with sickle cell disease at a major academic medical center

"Abstract The University of North Carolina at Chapel Hill is a tertiary-care, academic university hospital, and a major referral center for patients throughout North Carolina. " This 700-bed Level 1 trauma center transplants more than 22,000 RBC units to patients each year. The UNC sickle cell center, which opened in 1980 and continues today in collaboration with our neighboring institution Duke University Medical Center, has been designated as part of the National Institutes of Health comprehensive sickle cell center. Several of the physicians are dedicated to the care of pediatric and adult patients with SCD, as well as on transfusion management of these patients and recruiting African American blood donors. This essay explores the methods of this group for transfusion management of patients with SCD, as well as other aspects of the organization's transfusion medicine research.

Source link: https://doi.org/10.21307/immunohematology-2019-364

The Effects of Sickle Cell Disease on the Quality of Life: A Focus on the Untold Experiences of Parents in Tanzania

"Tanzania is one of the world's top five countries with a high incidence of sickle cell disease. " Despite the fact that the effects of SCD on quality of life have been documented in other countries, including N. . . . ia and the United States of America, few are known from Tanzania. This report, therefore, focused on investigating the effects of SCD on the quality of life among children living with SCD and their parents. The study used a qualitative method to interview prospective parents of children with SCD and who have used hydroxyurea for more than three years. In Dar-es-salaam, Tanzania, intensive interviews were conducted with 11 parents of children with SCD at the Muhimbili University of Health and Allied Sciences. Children with SCD and their parents suffer with emotional, financial, and physical consequences of the illness.

Source link: https://doi.org/10.3390/ijerph19116871

Transfusion practices for patients with sickle cell disease at major academic medical centers participating in the Atlanta Sickle Cell Consortium

More than 2600 pediatric and adult patients with sickle cell disease in the metropolitan Atlanta, Georgia, area treated by four major hospitals, each providing comprehensive health services 24 hours a day, 7 days a week. Although exact figures are uncertain and are now under scrutiny, alloimmunization occurs infrequently, with the exception of chronically transfused SCD patients, who account for the minority of active SCD patients.

Source link: https://doi.org/10.21307/immunohematology-2019-145

Occurrence of antibodies to low-incidence antigens among a cohort of multiply transfused patients with sickle cell disease

"The results from 137 recurrently transfused patients with SCD were chosen on the basis of transfusion activity from the 2009 calendar year. " One or two antibodies to AA-LIAs were found by 13 patients, and a total of 17 antibodies against these AA-LIAs were produced by these patients. Considering the possibility of antibodies in multiply transfused patients with SCD, it is justified. ".

Source link: https://doi.org/10.21307/immunohematology-2019-188

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