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[SAS-MX Acid Hb]. - Agentúra Harmony vos

[SAS-MX Acid Hb]. - Agentúra Harmony vos

[SAS-MX Acid Hb]. - Agentúra Harmony vos

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<strong>SAS</strong>-<strong>MX</strong> ACID HB-10The most common haemoglobin abnormalities:Sickle Cell TraitThis is a heterozygous state showing <strong>Hb</strong>A and <strong>Hb</strong>S and a normal amount of <strong>Hb</strong>A 2 on cellulose acetate.Results on citrate agar show haemoglobins in the <strong>Hb</strong>A and <strong>Hb</strong>S migratory positions (zones).Sickle Cell AnaemiaThis is a homozygous state showing almost exclusively <strong>Hb</strong>S, although a small amount of <strong>Hb</strong>F may alsobe present.Sickle-C DiseaseThis is a heterozygous state demonstrating <strong>Hb</strong>S and <strong>Hb</strong>C.Sickle CeIl-Thalassaemia DiseaseThis condition shows <strong>Hb</strong>A, <strong>Hb</strong>F, <strong>Hb</strong>S, and <strong>Hb</strong>A 2 .In Sickle Cell b o -Thalassaemia <strong>Hb</strong>A is absent.In Sickle Cell b + -Thalassaemia <strong>Hb</strong>A is present in reduced quantities.Thalassaemia-C DiseaseThis condition shows <strong>Hb</strong>A, <strong>Hb</strong>F and <strong>Hb</strong>C.C DiseaseThis is a homozygous state showing almost exclusively <strong>Hb</strong>CThalassaemia MajorThis condition shows <strong>Hb</strong>F, <strong>Hb</strong>A and <strong>Hb</strong>A 2 .LIMITATIONSSome abnormal hemoglobins have similar electrophorotic mohilities and must be differentiated byother methodologies.Further testing required:1. Globin chain analysis (both acid and alkaline) and structural studies may be necessary in order topositively identify some of the more rare haemoglobins.2. Anion exchange column chromatography is the most accurate method for quantitating <strong>Hb</strong>A 2 .Helena BioSciences Sickle-Thal Quik Column Method (Cat. No. 5334) for quantitation of <strong>Hb</strong>A 2 inthe presence of <strong>Hb</strong>S, or the Helena BioSciences Beta-Thal <strong>Hb</strong>A 2 Quik Column Procedure(Cat. No. 5341) are recommended. <strong>Hb</strong>A 2 quantitation is one of the most important diagnostictests in the diagnosis of B-thalassaemia trait.3. Low levels of <strong>Hb</strong>F (1-10%) may be accurately quantitated by radial immunodiffusion using theHelena BioSciences <strong>Hb</strong>F-QuiPlate Procedure (Cat. No. 9325).REFERENCE VALUESAt birth, the majority of haemoglobin in the erythrocytes of the normal individual is foetal haemoglobin,<strong>Hb</strong>F. Some of the major adult haemoglobin, <strong>Hb</strong>A, and a small amount of <strong>Hb</strong>A 2 , are also present.At the end of the first year of life and through adulthood, the major haemoglobin present is <strong>Hb</strong>A withup to 3.7% <strong>Hb</strong>A 2 and less than 2% <strong>Hb</strong>F.5English

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