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 Mild neutropenia in 20%
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 Blackfan Anemia 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 Increased Hb F
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 Non-responders
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

13. DBA
TEC
Age
Infancy
1-3y/o
Inherited?
Acquired
Antecedent illness No
Viral illness
Abnormal facies/anomalies
Yes: 25-50%
RBC Adenosine Deaminase
High
Normal
MCV
(?high in recovery)
Hgb F
(?high in recovery