Not used in treatment of thalassemia:
Correct Answer: Iron therapy
Description: Ans. b (Iron therapy). (Ref. Harrison, Principles of Medicine, 18th/Ch. 104)# Iron therapy is infact contraindicated in thalassemic patients on transfusion, as they are likely to have iron overload.Many patients with Beta-thalassemia major require chronic hypertransfusion therapy designed to maintain a hematocrit of at least 27-30% so that erythropoiesis is suppressed. Splenectomy is required if the annual transfusion requirement (volume of RBCs per kilogram of body weight per year) increases by >50%. Folic acid supplements may be useful. Vaccination with Pneumovax in anticipation of eventual splenectomy is advised, as is close monitoring for infection, leg ulcers, and biliary tract disease. Many patients develop endocrine deficiencies as a result of iron overload. Early endocrine evaluation is required for glucose intolerance, thyroid dysfunction, and delayed onset of puberty or secondary sexual characteristics.Patients with Beta-thalassemia intermedia exhibit similar stigmata but can survive without chronic hypertransfusion.Persons with alpha-thalassemia trait may exhibit mild hypochromia and microcytosis usually without anemia. HbA2 and HbF levels are normal. Affected individuals usually require only genetic counseling. HbH disease (in which three loci deleted on the contrary to Hb Barts, in which all four loci are deleted) resembles -thalassemia intermedia, with the added complication that the HbH molecule behaves like moderately unstable hemoglobin. Patients with HbH disease should undergo splenectomy if excessive anemia or a transfusion requirement develops. Oxidative drugs should be avoided. Iron overload leading to death can occur in more severely affected patients.
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