Pediatric extramedullary hematopoiesis

What are the signs and symptoms of pediatric extramedullary hematopoiesis?

• Enlarged spleen
• Enlarged liver
• Nodules on the skin that often have a bluish tint

How is pediatric extramedullary hematopoiesis treated?

Extramedullary hematopoiesis is a symptom of three conditions (thalassemia, congenital dyserythropoietic anemia and sideroblastic anemia).

Treatments include:

• Blood transfusions - Children who have severe forms of the conditions that cause extramedullary hematopoiesis need ongoing blood transfusions and careful monitoring.
  • In this procedure, we give patients healthy blood from a donor. A red blood cell transfusion lasts between three and four hours and has to be repeated monthly.
  • Blood transfusions turn off the body’s effort to make blood, which reverses the extramedullary hematopoiesis.
• Surgery - When a child's spleen is enlarged and causes difficulty with blood transfusions, a surgeon may need to remove it. Surgeons often use minimally invasive surgical techniques (laparoscopy) that cause less scarring and allow the child to recover faster.
  • Because the spleen is important in fighting some bacterial infections, your doctor will need to give additional vaccinations before removing the spleen.
  • A child can have an active life without a spleen, but they will be at higher risk for infections.
• Bone marrow transplant - replaces your child’s diseased bone marrow with healthy, new cells from an appropriate donor. The bone marrow donor needs to be a close match to your child. The new bone marrow starts creating normal, healthy red blood cells.