Key Features of the Paper
STUDY AIM
To reach conclusions about an association between Wilms’ tumor gene (WT1), expression levels and high risk APL.
Methods
This is a report an individual case study of a 42-year old male patient with high risk APL with expressed high level of WT1harboring a complex karyotype.
Results
This is a case of APL with high white blood cell counts in blood tests and hypogranular variant morphology in bone marrow, together with fms-like tyrosine kinase-3 with internal tandem duplication mutations (FLT3-ITD), and bcr-3 isoform of PML-RARα.
High level of Wilms’ tumor gene (WT1) were detected in marrow blasts, through the reverse transcription polymerase chain reaction (RT-PCR).
This patient successively received a combined treatment regimen consisting of hydroxycarbamide, arsenic trioxide and idarubicin plus cytarabine, which ultimately enabled complete remission.
Unfortunately, he subsequently died of sudden massive hemoptysis because of pulmonary infection.
Conclusion
There is controversy as to whether high WT1 expression suggests a poor prognosis for AML. However, the authors believe that high WT1 expression in APL cases indicates an adverse prognosis and should be considered in APL risk stratification. Although the clinical data for further validation is far from plentiful, WT1 inhibitor or WT1-specific cytotoxic cell therapy may be promising in cases of high-risk APL with WT1 overexpression.