Chapter 11: Hemoglobinopathies: Thalassemia

Question 1

B-Thalassemia

Answer 1

(Beta)-B Thalassemia is most common

-transfusion dependent and have a marked hepatosplenomegaly and bone changes secondary to increased hematopoiesis

Question 2

Thalassemia

Answer 2

named and classified by the type of globin chain that is inadequately produced

• the defective chains damage the developing RBCs by oxidative injury, resulting in chronic hemolysis and, in iron overload
• An inherited blood disorder characterized by the formation of abnormal form of hemoglobin

Question 3

Management

Answer 3

• close maternal and fetal surveillance
• ongoing consultations and appropriate treatment by maternal-fetal medicine and hematology specialists
• iron supplementation only if necessary

Question 4

Findings Associated

Answer 4

• high RBCs count
• moderate to marked microcytosis
• peripheral smear that resembles iron deficiency
• or may have severe overload of iron because of the increased iron absorption that results from ineffective erythropoiesis