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

A 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.