title: Acute WT1-positive promyelocytic leukemia with hypogranular variant morphology, bcr-3 isoform of PML-RARα and Flt3-ITD mutation: a rare case report.
abstract: 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.
  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.
  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.
authors.affiliation: MD, PhD. Professor, Department of Hematology, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Xi
authors.initials: X
authors.lastname: Zhang
authors.affiliation: MD. Attending Physician, Department of Hematology, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Cheng
authors.initials: C
authors.lastname: Yang
authors.affiliation: MD. Affiliated Professor, Department of Hematology, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Xiangui
authors.initials: X
authors.lastname: Peng
authors.affiliation: MD, PhD. Full Professor, Department of Hematology, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Xinghua
authors.initials: X
authors.lastname: Chen
authors.affiliation: MD, PhD. Assistant Professor, Department of Hematology, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Yimei
authors.initials: Y
authors.lastname: Feng
mesh_terms: D000328:Adult; D016162:Genes, Wilms Tumor; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D008297:Male; D009154:Mutation; D016133:Polymerase Chain Reaction; D011379:Prognosis; D051562:Proto-Oncogene Proteins c-bcr; D012307:Risk Factors; D051941:fms-Like Tyrosine Kinase 3 / Q000235:genetics*
chemical_list: D051941:fms-Like Tyrosine Kinase 3; C496613:BCR protein, human; D051562:Proto-Oncogene Proteins c-bcr