Bibliographic Details
| Title: |
Acute WT1-positive promyelocytic leukemia with hypogranular variant morphology, bcr-3 isoform of PML-RARα and Flt3-ITD mutation: a rare case report |
| Authors: |
Zhang, Xi, Yang, Cheng, Peng, Xiangui, Chen, Xinghua, Feng, Yimei |
| Source: |
Sao Paulo Medical Journal. April 2017 135(2) |
| Publisher Information: |
Associação Paulista de Medicina - APM, 2017. |
| Publication Year: |
2017 |
| Subject Terms: |
Leukemia, promyelocytic, acute, Fms-like tyrosine kinase 3, WT1 proteins, Prognosis, Lung diseases, fungal |
| More Details: |
CONTEXT: Acute promyelocytic leukemia (APL) accounts for 8% to 10% of cases of acute myeloid leukemia (AML). Remission in cases of high-risk APL is still difficult to achieve, and relapses occur readily. CASE REPORT: Here, we describe 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. Most importantly, we detected high level of Wilms’ tumor gene (WT1) in marrow blasts, through the reverse transcription polymerase chain reaction (RT-PCR). To date, no clear conclusions about an association between WT1 expression levels and APL have been reached. 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: Based on our findings and a review of the literature, abnormal functioning of WT1 may be a high-risk factor in cases of APL. Further studies aimed towards evaluating the impact of WT1 expression on the prognosis for APL patients are of interest. |
| Document Type: |
report |
| File Description: |
text/html |
| Language: |
English |
| ISSN: |
1516-3180 |
| DOI: |
10.1590/1516-3180.2016.020104102016 |
| Access URL: |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31802017000200179 |
| Rights: |
info:eu-repo/semantics/openAccess |
| Accession Number: |
edssci.S1516.31802017000200179 |
| Database: |
SciELO |
