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A 9-mon th-old male patien t is referred by his pediatrician due to an abnormal blood count. Leu kocyte count is 7 , 300/f..Ll. hemoglobin is 7_/..j g/dl with MCV 1 09 fl. reticu-locytes is 1 . 0%, and platelet count is 58 1 .000/ f..Ll. The physical examination reveals no abnormal fi ndi ngs . The peripheral blood smear shows mild macrocytosis but is other-wise unremarkable. The i mage below shows the bone marrow aspirate specimen.
Diamond-Blackfan anemia or transient erythroblastopenia of childhood (TEC) are considered in the differential diagnosis. Which of the following choices is correct in describing the findings that would support a diagnosis ofDiamond-Blackfan anemia (DBA)?
- DBA is more likely to be diagnosed after 1 year of age, whereas TEC is diagnosed before age 1 in most cases
- TEC is more likely to be macrocytic
- HbF is usually increased in patients with DBA
- Erythrocyte adenosine demaminase (ADA) levels are elevated in TEC
Medicine
C. The image shows a normocellular marrow with decreased to absent red cell precursors. This could represent either DBA or TEC. Several factors can be used to differentiate these two disor-ders including the following: DBA usually occurs before age 1, the anemia is macrocytic, HbF is ele-vated, and erythrocyte ADA is elevated (Br J Haematol. 2008;142:859-876).
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