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Bart Information and Courses from MediaLab, Inc.

These are the MediaLab courses that cover Bart and links to relevant pages within the course.

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Alpha Thalassemia
Anemia in Alpha Thalassemia

In thalassemia there is often an excess production or accumulation of globin chains produced by genes that are not effected by the thalassemia deletion. In alpha thalassemia this may be seen as gamma chain tetramers (hemoglobin Bart's) in the unborn child and as beta chain tetramers (hemoglobin H) in adults. Tetramer accumulation often leads to red blood cell damage and hemolytic anemia.

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Alpha Thalassemia Major

Anemia is fatal.Red blood cell (RBC) count is increased.Hemoglobin (Hb) is severely decreased.Mean corpuscular volume (MCV) is decreased. Mean corpuscular hemoglobin concentration (MCHC) is decreased.Red cell distribution width (RDW) is increased.RBC morphology shows slight hypochromic microcytosis with codocytes, schizocytes, nucleated RBCs.Reticulocytes are increased.Hb electrophoresis demonstrates abnormal pattern on cord blood: Hb A - absentHb Bart's - 80-90%Hb Portland - 0-20%Bone marrow demonstrates marked erythroid hyperplasia.

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Alpha Thalassemia Intermedia

Anemia is moderate.RBC count is increased.Hb is moderately decreased.MCV is decreased. MCHC is decreased.RDW is increased.RBC morphology shows slight hypochromic microcytosis with codocytes, schizocytes, and basophilic stippling.Reticulocytes are moderately increased.Hb electrophoresis demonstrates abnormal patterns in both adults and neonates.Adults:HbA decreasedHbA2 decreasedHbF normal to decreasedHb H -2-40% (beta chain tetramers)Neonates: 10-40% Bart's (gamma chain tetramers)Hb H inclusions are frequently seen.Bone marrow demonstrates erythroid hyperplasia.

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Alpha Thalassemia Minor

Anemia is mild to absent.RBC count is increased.Hb is slightly decreased.MCV is decreased. MCHC is slightly decreased.RDW is normal to slightly increased.Red Blood Cell morphology shows slight hypochromic microcytosis.Reticulocytes are normal to slightly increased.Hb electrophoresis demonstrates a normal pattern in adults:Hb A - 97-98% Hb A2 - 1-2.5% Hb F - <1%. Neonates have 5-15% Bart's Hemoglobin (gamma chain tetramers).Hb H inclusions are rarely seen.Bone marrow demonstrates erythroid hyperplasia.

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Silent Carrier

Anemia is absent.RBC count is within normal limits.Hb is within normal limits.MCV is normal to slightly decrease.MCHC is normal to slightly decrease.RDW is within normal limits.Red Blood Cell morphology is normal.Reticulocytes are within normal limits.Hb electrophoresis demonstrates a normal pattern in adults:Hb A - 97-98%Hb A2 - 1-2.5% Hb F - < 1%. Neonates have 1-2% Bart's Hemoglobin (gamma chain tetramers).Hb H inclusions are rarely seen.Bone marrow is normal.

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Migration of Hemoglobin in Alkaline Electrophoresis

Of the hemoglobins normally present in an adult, Hb A migrates the fastest, followed by Hb F. Hb A2 moves only slightly from the point of origin near the cathode.Abnormal hemoglobins show the following migration patterns: Hb C migrates with Hb A2 near the cathode. Hb S lies between Hb A2 and Hb F. Hb H and Bart's hemoglobin are unstable and very fast moving placing them past Hb A and near the anode with Hb H being the fastest of the two.Relative migrations of hemoglobin variants on alkaline electrophoresis can be seen below.

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Beta Thalassemia
Migration of Hemoglobin in Alkaline Electrophoresis

Of the hemoglobins normally present in an adult, Hb A migrates the fastest, followed by Hb F. Hb A2 moves only slightly from the point of origin near the cathode.Abnormal hemoglobins show the following migration patterns:Hb C migrates with Hb A2 near the cathode.Hb S lies between Hb A2 and Hb F.Hb H and Bart's hemoglobin are unstable and very fast moving, with Hb H being the faster of the two. They are located nearer the anode past Hb A .Relative migrations of hemoglobin variants on alkaline electrophoresis can be seen below.

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