title: Acute Promyelocytic Leukemia: A Review and Discussion of Variant Translocations.
abstract: The majority of patients with acute promyelocytic leukemia (APL) manifest the t(15;17)(q24.1;q21.2) translocation; however, a minor but significant proportion of patients with APL harbor complex, cryptic, or variant translocations, which typically involve RARA. With the exception of ZBTB16/RARA, these variants have similar morphologic and immunophenotypic features as classic APL. Study of the variant forms of APL not only gives insight into the pathogenesis of APL but also allows us to understand the mechanism of retinoid therapy. It is important to identify these cryptic and variant translocations because certain variants, including ZBTB16/RARA and STAT5B/RARA, are resistant to treatment with all-trans retinoic acid, arsenic trioxide, and anthracyclines.
authors.affiliation: From the Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis.
authors.forename: Julia
authors.initials: J
authors.lastname: Adams
authors.forename: Mehdi
authors.initials: M
authors.lastname: Nassiri
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D003937:Diagnosis, Differential; D019008:Drug Resistance, Neoplasm; D006801:Humans; D051741:Kruppel-Like Transcription Factors / Q000235:genetics; D015473:Leukemia, Promyelocytic, Acute / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D000075722:Promyelocytic Leukemia Zinc Finger Protein; D018168:Receptors, Retinoic Acid / Q000235:genetics; D000072482:Retinoic Acid Receptor alpha; D050799:STAT5 Transcription Factor / Q000235:genetics; D014178:Translocation, Genetic
chemical_list: D000970:Antineoplastic Agents; D051741:Kruppel-Like Transcription Factors; D015514:Oncogene Proteins, Fusion; D000075722:Promyelocytic Leukemia Zinc Finger Protein; C000607071:RARA protein, human; D018168:Receptors, Retinoic Acid; D000072482:Retinoic Acid Receptor alpha; D050799:STAT5 Transcription Factor; C494095:STAT5B protein, human; C092037:ZBTB16 protein, human