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INFO VERTICAL CIRCO VINTAGE

Valeria Ortiz

Created on November 16, 2023

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beta thalassemia

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Beta thalassemia is a genetic disorder caused by point mutations in the beta-globin gene on chromosome 11 on hemoglobin , which is the oxygen-carrying protein in erythrocytes. This abnormal hemoglobin can lead to anemia.

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SYMPTOM S

Autosomal rececive Diseas

Two mutated copies of this gene, one from each parent, are needed to develop the disease

1. Severe Anemia 2. Growth retardation 3. Hepatosplenomegaly and lymphadenopathy 4. facial changes "squirrel face". 5. Increased risk of fracture 6. Widening of the medullary cavity and bone thinning (spiky hair sign) 7. Patient prone to infections 8. Increased risk of thrombosis

Blood smearS

Pathogenesis

1. Basophil stitch 2. Dianocytes or condocytes 3. Jolly Howell Bodies

Labs

1. Severe Anemia 2. Low MCV 3. Low MCH 4. Hypochromic and microlitic erythrocytes 5. Hypercellularity, erythroid hyperplasia

Electrophoresis

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Beta thalassemia Inermedia

The person has two mutated genes that code for reduced beta globin chain synthesis

Moderately severe anemia that usually requires usually requires regular blood transfusions

Beta thalassemia minor

If the person has just one mutated gene that codes for either reduced production or absent production8 of beta-globin chains.

Mild anemia usually does not require medical treatment.

Beta thalassemia Inermedia

The person has two β0 mutations then no beta globin chains are produced

Potentially fatal anemia