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Alpha Thalassemia: Clinical Syndromes and Classification

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Alpha Thalassemia: Clinical Syndromes and Classification

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Questions and Answers

What is the ratio of Alpha/Beta chain in Silent Carrier State of Alpha Thalassemia?

More than normal

Nearly normal (correct)

Cannot be determined

Significantly decreased

What is the most common cause of Hemoglobin H Disease?

Presence of only one gene producing alpha chains (correct)

Deletion of all four alpha genes

Deletion of two alpha genes

Deletion of three alpha genes

What is the typical MCV range in Alpha Thalassemia Trait (Alpha Thalassemia Minor)?

85-90 fL

80-85 fL

75-80 fL

70-75 fL (correct)

What is the percentage of Hemoglobin H in adults with Hemoglobin H Disease?

30-50% Signup and view all the answers

What can trigger a hemolytic crisis in individuals with Hemoglobin H Disease?

Infections, pregnancy, and exposure to oxidative drugs Signup and view all the answers

Why is it difficult to diagnose Silent Carrier State of Alpha Thalassemia?

Because there are no hematologic abnormalities Signup and view all the answers

What is the significance of taking a patient's individual history and family history in the diagnosis of thalassemia?

To understand the ethnic background and its relevance to the disease Signup and view all the answers

Which of the following physical examination findings is indicative of hemolysis?

Jaundice Signup and view all the answers

What is the characteristic pattern seen in a CBC blood film in thalassemia?

Microcytic, hypochromic Signup and view all the answers

What is the purpose of the Brilliant Cresyl Blue Stain in the diagnosis of thalassemia?

To detect Hemoglobin H precipitate Signup and view all the answers

Which of the following is a characteristic feature of thalassemia that differentiates it from iron deficiency anemia?

Elevated RBC count with markedly decreased MCV Signup and view all the answers

What is the purpose of the Acid Elution Stain in the diagnosis of thalassemia?

To detect Hemoglobin F Signup and view all the answers

What is the characteristic of Hemoglobin Lepore?

Gene crossovers between delta locus on one chromosome and beta locus on second chromosome Signup and view all the answers

What is the result of absence of alpha chains in Alpha Thalassemia?

Increase of gamma chains during fetal life Signup and view all the answers

What is the characteristic of Beta Thalassemia Minor?

Hb A is decreased, Hb A2 is normal, Hb F is increased Signup and view all the answers

What is the primary cause of alpha thalassemias?

Gene deletions in the alpha-globin gene Signup and view all the answers

What is the laboratory feature of Thalassemia Major?

Microcytic and hypochromic anemia with anisocytosis and poikilocytosis Signup and view all the answers

What is the potential future treatment for Thalassemia?

Bone marrow transplants Signup and view all the answers