Early Death in Two Patients with Acute Promyelocytic Leukemia Presenting the bcr3 Isoform, FLT3-ITD Mutation, and Elevated WT1 Level | Zendy

Marianna Greco | Zendy; Giovanni Caocci | Zendy; A Ledda | Zendy; Adriana Vacca | Zendy; Marcella Arras | Zendy; Ivana Celeghini | Zendy; Giorgio La Nasa | Zendy

Open Access

Early Death in Two Patients with Acute Promyelocytic Leukemia Presenting the bcr3 Isoform, FLT3-ITD Mutation, and Elevated WT1 Level

Author(s) -

Marianna Greco,

Giovanni Caocci,

A Ledda,

Adriana Vacca,

Marcella Arras,

Ivana Celeghini,

Giorgio La Nasa

Publication year - 2013

Publication title -

case reports in hematology

Language(s) - English

Resource type - Journals

eISSN - 2090-6560

pISSN - 2090-6579

DOI - 10.1155/2013/896394

Subject(s) - acute promyelocytic leukemia , medicine , gene isoform , mutation , tyrosine kinase , gene duplication , oncology , point mutation , tandem exon duplication , clinical significance , cancer research , gene , genetics , biology , retinoic acid , receptor

Despite major advances in the treatment of acute promyelocytic leukemia (APL), the problem of early death (ED) remains unsolved. Alongside the currently known clinical and hematological risk factors, prognostic significance has been attributed to internal tandem duplication mutations of the fms-like tyrosine kinase-3 (FLT3-ITD), hypogranular variant morphology, and the bcr-3 isoform of PML-RAR α . We describe premature death of two patients with the hypogranular variant of APL who presented remarkably high expression levels of Wilms' tumor gene (WT1). Our results point to WT1 as an important prognostic factor of ED that needs to be promptly evaluated in all newly diagnosed cases of APL.

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