Thalassemia

In beta thalassemia, which unpaired globin chains accumulate in excess causing membrane lipid oxidation and damage?

• Alpha

• Beta

• Delta

• Gamma

The alpha-globin gene cluster is on chromosome ___.

• 7

• 11

• 16

• 21

Which of these factors facilitates more effective delivery of oxygen from hemoglobin to the tissues?

• Low 2,3-bisphosphoglycerate (2,3-BPG)

• Carbon monoxide exposure

• Tissue acidosis

• Lower temperature

What is the predominant cause of anemia in beta thalassemia?

• Folic acid deficiency

• Ineffective erythropoiesis

• Iron overload

• Splenic sequestration

What is the underlying globin abnormality in Hb Bart's hydrops fetalis?

• Absence of a normally functioning alpha globin gene.

• Deletion of 1 alpha-globin gene

• Deletion of three alpha-globin genes

• Two alpha-globin genes are poorly expressed

An asymptomatic 21-year-old female was referred by her company physician for preemployment clearance (BPO company). Routine CBC showed the ff findings: hemoglobin 118, low MCV and MCH, ferritin of 180 ng/ml, and no increase in HbA2 on hemoglobin electrophoresis. What is the most likely diagnosis?

• Beginning iron deficiency

• Alpha thalassemia trait

• Beta thalassemia

• Inconclusive findings

Which of the following is an intravenous iron chelator?

• Deferoxamine

• Deferasirox

• Deferiprone

• None of the above

Which of these can contribute to iron overload among patients with thalassemia?

• Medicinal iron intake

• Regular blood transfusion

• Erythroid hyperplasia

• Inappropriately low hepcidin level

O2 loading and unloading occur when heme iron is in its reduced ferrous form. True or False?

• True

• False

What is the most common acute event in sickle cell disease?

• Acute chest syndrome

• Acute painful episodes

• Hip osteonecrosis

• Leg ulcers