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Bleeding and Clotting Disorders

Spherocytosis in infants

10/09/2007

Question:

My daughter was giagnoised with HS when she was 7 weeks old she is now 9 months old and has had 5 tranfusions. When she was first diagnoised her blood smear showed that 40% of her cells were abnormal, now recent smears show that 60% of her cells are abnormal. Why do these cells counts countine to increase? What does this mean for her now and in the future? On avarage in between transfusions her heomoglobin stays around a 7 to a 7.5, will this cause any lasting problems for her in regards to her vital organs? Can she countine to have transfusion this frequently without there being an adverse effect?

Answer:

Hereditary spherocytosis is a congenital disorder in which the red cells are more fragile and can be hemolyzed or ruptured in the vessel.  In patients with severe enough hemolysis, splenectomies are sometimes done to help with this.  I'm sure you've already seen a pediatric hematologist, so I would discuss this with them to see if it is something they are considering. Long-term transfusions can cause some problems related to iron overload and related issues, which is why splenectomy is considered.

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Response by:

Spero R Cataland, MD Spero R Cataland, MD
Associate Professor of Clinical Internal Medicine
College of Medicine
The Ohio State University