Sickle cell preparation

Citation Julie Kanter , Robert I. Liem , Françoise Bernaudin , Javier Bolaños-Meade , Courtney D. Fitzhugh , Jane S. Hankins , M. Hassan Murad , Julie A. Panepinto , Damiano Rondelli , Shalini Shenoy , John Wagner , Mark C. Walters , Teonna Woolford , Joerg J. Meerpohl , John Tisdale; American Society of Hematology 2021 guidelines for sickle cell disease: stem cell transplantation. Blood Adv 2021; 5 (18): 3668–3689. doi: Download citation file: • • • • • • • • • Background: Sickle cell disease (SCD) is a life-limiting inherited hemoglobinopathy that results in significant complications and affects quality of life. Hematopoietic stem cell transplantation (HSCT) is currently the only curative intervention for SCD; however, guidelines are needed to inform how to apply HSCT in clinical practice. Objective: These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and health professionals in their decisions about HSCT for SCD. Methods: The multidisciplinary guideline panel formed by ASH included 2 patient representatives and was balanced to minimize potential bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including performing systematic evidence reviews (through 2019). The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment...

Sickle cell anemia is an inherited disorder that leads to the production of an abnormal type of Hemoglobin is the protein in red blood cells (RBCs) that binds to oxygen in the lungs and carries it to tissues throughout the body.Typically, hemoglobin A (Hb A, adulthemoglobin) makes up most of the hemoglobin found in normal RBCs in adults, with small amounts of hemoglobin A2 and hemoglobin F. Before babies are born, they normally produce large amounts of hemoglobin F (Hb F, fetalhemoglobin), which is then replaced by Hb A as the predominant hemoglobin shortly after birth. Sickle cell disease is an inherited condition, passed from parents to children. Inheriting mutationsin thegenesthat code for the production of hemoglobin can lead to abnormal types of hemoglobin (variants),such as Hb S and hemoglobin C (Hb C). Hemoglobin Cis one of the more common hemoglobin variants and may cause no symptoms or mild illness. • Sickle cell disease and anemia—a person who inherits two abnormal gene copies (alleles), one of which is a Hb S gene, has sickle cell disease(i.e., a person who has one Hb S gene copy and one Hb C gene copy has sickle cell disease.) A person who inherits two Hb S gene copies (one from each parent;homozygous) has sickle cell anemia, the most common and serious for of sickle cell disease. • Sickle cell trait (carrier)—a person who inherits one normal hemoglobin gene copy from one parent and a Hb S gene copy from the other parent (heterozygous) has sickle cell trait and...

Diagnosis A blood test can check for the form of hemoglobin that underlies sickle cell anemia. In the United States, this blood test is part of routine newborn screening. But older children and adults can be tested, too. In adults, a blood sample is drawn from a vein in the arm. In young children and babies, the blood sample is usually collected from a finger or heel. The sample is then sent to a laboratory, where it's screened for the sickle cell form of hemoglobin. If you or your child has sickle cell anemia, your doctor might suggest other tests to check for possible complications of the disease. If you or your child carries the sickle cell gene, you'll likely be referred to a genetic counselor. Assessing stroke risk A special ultrasound machine can reveal which children have a higher risk of stroke. This painless test, which uses sound waves to measure blood flow in the brain, can be used in children as young as 2 years. Regular blood transfusions can decrease stroke risk. Tests to detect sickle cell genes before birth Sickle cell disease can be diagnosed in an unborn baby by sampling some of the fluid surrounding the baby in the mother's womb (amniotic fluid). If you or your partner has sickle cell anemia or the sickle cell trait, ask your doctor about this screening. Treatment Management of sickle cell anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications. Treatments might include medications and blood transfusions. For so...