Due to recent advances in immunosuppressive therapy, the prognosis for apla
stic anemia (AA) has improved dramatically. As more patients with this dise
ase have been surviving longer, the number of AA patients who develop myelo
dysplastic syndrome (MDS) or leukemia has increased. However, it is unclear
whether the development of this type of late clonal disorder is related to
a particular therapy, and whether hypoplastic MDS can be differentiated fr
om AA at diagnosis. Although there are no definitive answers to these quest
ions, some forms of AA and MDS are likely within the same spectrum of disea
se. Thus, when treating AA, it is essential to evaluate various clinical an
d laboratory information, including cell morphology and chromosomal aberrat
ion. This paper discusses acute myelocytic leukemia (AML) and MDS in childr
en diagnosed as having AA, and it presents problems associated with the dia
gnosis of this disease.