Sickling test

Homozygous hemoglobin (Hb) S (sickle cell disease) is a serious chronic hemolytic anemia most commonly found in those of African or Middle Eastern descent. Hb S is freely soluble when fully oxygenated; when oxygen is removed, polymerization of the abnormal hemoglobin occurs, forming tactoids that are rigid and deformed cells. This leads to sickling of the cells, hemolysis, and many other complications. Heterozygous Hb S (sickle cell trait) is the most common hemoglobinopathy in the United States. This condition is present in about 8% of African Americans. Usually, Hb S trait exhibits no clinical or hematological effects. A small fraction of people with sickle cell trait have recurrent hematuria. A positive test is presumptive evidence for hemoglobin (Hb) S (sickle cell disease) . However, rare sickling Hbs such as Hb C-Harlem (C-Georgetown) and Hb I will alsoproduce a positiveresult. This test only detects the presence of Hb S. It cannot differentiate sickle cell trait (heterozygous Hb S) from sickle cell disease (homozygous Hb S), or Hb S in combination with other abnormalities (eg, S/C, S/D, S/G, S/E, S/beta-thalassemia, S/O-Arab, S/New York, and C-Georgetown trait). The use of packed red blood cells (RBC) instead of whole blood significantly reduces false negatives due to anemia and false positives due to hypergammaglobulinemia (eg, multiple myeloma). False positives can occur due to large numbers of nucleated RBC. False negatives can occur due to an insufficient quanti...

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...

No. People with sickle cell anemia (Hgb S/S) have the hemoglobin S gene mutation on both of their beta globin genes, whereas people with sickle cell trait (Hgb A/S) have the mutation on only one beta globin gene. People with sickle cell trait are considered carriers and are usually asymptomatic. However, sickle cell formation leading to vascular occlusion can occur during high fever and under conditions of significant hypoxia. Differentiating sickle cell anemia from the trait is important for clinical management and genetic counseling of the individual. No. Sickle cell disease refers to any condition that causes a sickling disorder. Sicklecell anemia is one type of sickle cell disease. It is caused by the presence of a Hgb S on both beta globin genes. Other types of sickle cell disease are caused by presence of Hgb S on one beta globin gene and Hgb C, Hgb D-Punjab (D-Los Angeles), Hgb O-Arab, Hgb C-Harlem, Hgb Lepore, or beta thalassemia on the other beta globin gene. The sickle cell solubility test is a qualitative test based on the relative insolubility of hemoglobin S compared to other hemoglobin variants. A positive test indicates the presence of hemoglobin S or a non-S sickling hemoglobin. Results should be confirmed by high-performance liquid chromatography (HPLC) (Hemoglobinopathy Evaluation, test code 35489[X]). 1 A positive test is consistent with sickle cell trait, sickle cell anemia and hemoglobin S in combination with another hemoglobin variant, e.g. hemoglobin...

Sickle Cell Procedures and Interpretation Preparation of working sickle cell buffer Add the entire contents of one vial of Sickle Cell Lysing Reagent to 50ml of Sickle Cell Buffer and mix completely by swirling. The working buffer may be used for 30 days from date of preparation if stored at 2° to 8° C. Assay Protocol • Transfer 2.0ml of the Working Sickle Cell Buffer to a 12 x 75 mm tube. • Add 20μl of well-mixed whole blood or control specimen and mix thoroughly and gently. If testing a specimen with a hemoglobin below 7 g/dl, add 40 μl of whole blood. • Allow to stand for 5 minutes but no longer than 30 minutes at room temperature (15° to 30° C). • Read results by looking through the test tube at the Line Scale (see below). Hold the test tube upright and perpendicular approximately 3 cm from the Line Scale. Adequate illumination is necessary. Interpretation • Reactive - If any sickling hemoglobin is present, the solution will be sufficiently turbid to prevent reading the Line Scale through the test tube. • Nonreactive - If a sickling hemoglobin is not present, the solution will be clear enough to allow the Line Scale to be seen through the test tube.

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