Hidden mastocytosis in acute myeloid leukemia with t(8;21)(q22;q22)

Am J Clin Pathol. 2013 Oct;140(4):525-35. doi: 10.1309/AJCP1Q0YSXEAHNKK.

Abstract

Objectives: To assess the frequency of systemic mastocytosis (SM) in a large series of acute myeloid leukemia (AML) with t(8;21)(q22;q22).

Methods: We retrospectively characterized 40 bone marrow aspirate smears and biopsy specimens from patients with AML with t(8;21) for the presence of SM. Cases were assessed for mast cell morphology and immunohistochemistry, as well as KIT exon 8 and 17 mutational assessment by reverse transcription polymerase chain reaction.

Results: Four patients met criteria for SM, 1 met criteria for myelomastocytic leukemia, and 8 demonstrated the benign finding of mast cell hyperplasia.

Conclusions: We recommend examining all cases of AML with t(8;21) for the presence of SM via morphology, immunophenotyping, and KIT mutational analysis studies.

Keywords: AML with t(8;21); CD25; D816V; Exon 17 KIT mutations; Myelomastocytic leukemia; N822K; Systemic mastocytosis; Tryptase.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Bone Marrow Cells / pathology
  • Child
  • Child, Preschool
  • Chromosomes, Human, Pair 21*
  • Chromosomes, Human, Pair 8*
  • DNA Mutational Analysis
  • Female
  • Humans
  • Hyperplasia
  • Leukemia, Myeloid, Acute / complications
  • Leukemia, Myeloid, Acute / genetics
  • Leukemia, Myeloid, Acute / metabolism
  • Leukemia, Myeloid, Acute / pathology*
  • Male
  • Mast Cells / pathology
  • Mastocytosis, Systemic / complications
  • Mastocytosis, Systemic / genetics
  • Mastocytosis, Systemic / metabolism
  • Mastocytosis, Systemic / pathology*
  • Middle Aged
  • Proto-Oncogene Proteins c-kit / genetics
  • Retrospective Studies
  • Translocation, Genetic*
  • Young Adult

Substances

  • Proto-Oncogene Proteins c-kit