Tiff
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An 8 year old boy has a chronic history of severe hemolytic anemia, hepatoslenomegaly, and maxillary overgrowth. He has received blood transfusions since early infancy but has not received a trasfusion in over 4 months. A hemoglobin electrophoresis shows marked elevation of HbF, increased HbA2, and absence of HbA1. Which of the following diagnoses is most consistent with this patient's electrophoresis?
A. alpha-Thalassemia minor
B. beta-Thalassemia major
C. beta-Thalassemia minor
D. Glucose-6-phosphate dehydrogenase deficiency
E. HbH disease
F. RBCs containing hemoglobin Barts
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| jean robert
Forum Guru
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B?
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| Idlehands
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C. beta-Thalassemia minor
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| new_n_lost
Politically InCorrect
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B. beta-Thalassemia major
ref Papi pg 206 First side note.
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| Tiff
Forum Guru
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 The correct answer is B. (B-thalassemia major).
B-thalassemia minor is incorrect because with minor, there are reduced levels of HbA1. In major, HbA1 is absent. (HbA1 is composed of alpha-2/beta-2)
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| Misrati
Forum Junior
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I would go with ( B-thalassemia Major ) , due to the following :
1- child age
2-Hb electro = mainly Hb F
3-ITS OBVIOUS that the child's condition severe since early infancy and he has been on Bl.transfusion since then ..that he needs for ever .
PS : HOWEVER ,even Hb A2 is increased here over time that may mislead us to B-th triat which has Hb A2 increased to compensate the status , but Hb F wouldn't be sky high , nonetheless the case senario much mor benign unless the child had some sort of stress ..
its a good question " appreciated"
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