62.6k views
2 votes
8mo girl has RBC transfusion from anemia. Hemoglobin electrophoresis shows 5% HbA2, 95% HbF. What dx, what supportive meds to improve LT survival?

User Rajbir
by
8.1k points

1 Answer

6 votes

Final answer:

The 8-month-old girl likely has beta-thalassemia major, a genetic disorder impairing hemoglobin production. Treatments include regular blood transfusion, iron chelation therapy, and folic acid supplements. Bone marrow transplantation might improve long-term survival.

Step-by-step explanation:

The description provided suggests a diagnosis of beta-thalassemia major or Cooley's anemia. This is a genetic disorder that impairs the body's ability to produce hemoglobin. In this case, the high HbF (fetal hemoglobin) percentage is compensating for the insufficient HbA (adult hemoglobin), a common characteristic of beta-thalassemia major. For supportive medications, treatments often include blood transfusions and iron chelation therapy to maintain normal hemoglobin levels and remove excess iron from the body. The administration of folic acid supplements is also common in these cases because these patients often have high metabolic requirements. Furthermore, a possible long-term survival improver could be bone marrow transplantation, although it carries significant risks.

Learn more about Beta-thalassemia Major

User Renaud
by
8.1k points

Related questions

1 answer
0 votes
82.4k views
1 answer
0 votes
58.3k views