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A 2 1 -year-old man is seen in the hematology clinic For evaluation of pancytopenia. The onset was noted at age 1 2 . and the course has been progressive. Current leukocyte count is 2.1 00/ JLL. hemoglobin 8.LJ g/dl with MCV 98 FL. and platelet count 29.000/ JLL. Reticulocyte percen tage is decreased at 1 .2% of red blood cel ls. On physical exam. he is noted to have short stature and multiple cafe-au-lait spots. While shaki ng his hand. you note that he has no thumb. The peri pheral blood smear and bone marrow biopsy are shown below.
What treatment approach is warranted in patients with FA?
- Observation, followed by androgen administration with the onset of moderate bone marrow failure
- Bone marrow transplantation (BMT) at the time of diagnosis if there is an appropriate donor available
- Observation, followed by the use of hematopoietic growth factors
- Systemic chemotherapy at the time of diagnosis
Medicine
A. Patients with mild bone marrow failure may be observed closely. With the onset of mod-erate bone marrow failure (ANC 500 to 1 ,000, platelets 30,000-50,000/ JLL, and hemoglobin < 8 g/dL), androgens are given if BMT is not desirable or if there is not an appropriate donor. Treatment with BMT at the time of diagnosis has not been shown to improve outcomes.
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