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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.
What statement is true regarding the prognosis and treatment ofDiamond-Blackfan anemia?
- Survival at age 40 is less than 30%
- Chemotherapy is considered second-line treatment
- Matched sibling stem cell transplant results have been disappointing
- Spontaneous remissions occur in 20% of patients
- Corticosteroids should only be used intermittently
Medicine
D. About 20% of patients have been reported to have spontaneous remission of DBA. The remissions are most likely to occur before age 10, and most remissions are durable. Chemotherapy does not have a role in the treatment of DBA. Matched sibling stem cell transplant is often successful and is considered a first-line therapy. Corticosteroids have been a mainstay in the treatment of DBA for decades and are often used for a prolonged period, which makes monitoring for side effects a major aspect of therapy (Br f Haematol. 2008;142:859-876).
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