AML with inv(3)(q21q26.2) or t(3;3)(q21;q26.1); (RPN1-EVI1)

10/10/12

  • Primarily in adults

  • Commonly associated with myelodysplastic findings, particularly uninucleated or binucleated micromegakaryocytes

  • Peripheral blood often with thrombocytosis and giant agranular platelets

  • Blasts can be myeloid, monocytic or megakaryoblastic

  • Blasts often CD7+

  • Cytogenetics: often cryptic and can be easily missed by conventional cytogenetic assays, better detected by FISH for EVI-l rearrangement; often associated with -7

  • EVI-1, an antagonist of GATA-1

  • Poor prognosis

REFERENCES
  • Hematopathology, 2011. By Elaine Sarkin Jaffe, MD, Nancy Lee Harris, James Vardiman, MD, Elias Campo, MD and Daniel MD Arber, MD