MRCP UK EXAMINATIONS

ABDOMINAL

THALASSEMIA

Question [CLICK ON ANY CHOICE TO KNOW THE RIGHT ANSWER]
The predominant hemoglobin present in b0-thalassemia major is:
A
Hb A
B
Hb A2
C
Hb F
D
Hb C
Explanation: 

Detailed explanation-1: -The Hb pattern in beta-thalassemia varies according to beta-thalassemia type. In beta0 thalassemia, homozygotes HbA is absent and HbF constitutes the 92-95% of the total Hb. In beta+ thalassemia homozygotes and beta+/beta0 genetic compounds HbA levels are between 10 and 30% and HbF between 70-90%.

Detailed explanation-2: -Beta-thalassemia major typically shows markedly elevated HbF (30-to-greater than 95%) with normal to mildly elevated HbA2. The distinction between beta-thalassemia major and intermedia is a clinical one and does not have diagnostic laboratory findings.

Detailed explanation-3: -Patients with -thalassemia major have elevated Hb A2 and Hb F (although Hb F may be normal in some individual).

Detailed explanation-4: -Sickle cell beta thalassemia (Hb S/ Th) is an inherited form of sickle cell disease that affects red blood cells both in the production of abnormal hemoglobin, as well as the decreased synthesis of beta globin chains.

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