November 18, 2010

1. November 18, 2010

2. Transient Erythroblastopenia of Childhood

3. Transient Erythroblastopenia of Childhood • Most common childhood acquired red cell aplasia • Previously healthy children • Age 1-3y/o

4. Etiology • Poorly understood • Transient immunologic suppression of erythropoiesis • Often follows viral illness

5. Lab findings • Severe anemia • Reticulocytopenia • However, 10% are in recovery at time of diagnosis • Mild neutropenia in 20% • Platelets normal or elevated • MCV normal • Hgb F levels normal

6. Treatment • Nearly all recover 1-2 mos • RBC transfusion may be necessary • Hgb < 5 • Significant symptoms • Cardiovascular compromise • If > 1 transfusion needed, consider another dx • Steroids NOT useful

7. Diamond BlackfanAnemia vs. Transient Erythroblastopenia

8. Diamond Blackfan • Congenital hypoplastic anemia • Dx 1st year of life • Average age 3mos • Sporadic or familial • Apoptosis of erythroid progenitor cells • Increased circulating erythropoietin

9. DBA: Clinical Manifestations • May appear pale as neonate • Profound anemia (symptomatic) by 6m/o • Congenital anomalies in 50% • Short stature • Snub nose, wide-set eyes, thick upper lip • “Intelligent Facies” • Thumb abnormalities • Cardiac and Eye abnormalities

10. DBA: Lab findings • Macrocytosis • Normal Folic Acid and B12 levels • NO hypersegmentation of neutrophils • Increased Hb F • Erythrocyte adenosine deaminase (ADA) increased • Occasionally low plts or WBCs • Reticulocytopenia

11. DBA: Treatment • Prednisone • 75% respond initially • Mechanism unknown • Non-responders • Transfusion q4-8wks • Stem cell transplant as last resort

12. Prognosis • Survival 40yrs • Complications of stem cell transplant, corticosteroids, iron overload • ?Premalignant Syndrome? • AML • Myelodysplasia • Osteosarcoma