Juvenile myelomonocytic leukemia with prominent CD141+ myeloid dendritic cell differentiation

Hum Pathol. 2017 Oct:68:147-153. doi: 10.1016/j.humpath.2017.03.025. Epub 2017 Apr 13.

Abstract

Myeloid malignancies showing CD141+ myeloid dendritic cell (MDC) differentiation have not been documented. Here, we describe a patient with juvenile myelomonocytic leukemia in which a prominent CD141+ cell population was identified most consistent with CD141+ MDCs based on phenotypic similarity with normal CD141+ MDCs. Molecular studies demonstrated a KRAS mutation. The findings from the spleen and bone marrow are described. This is the first well-documented demonstration of CD141+ MDC differentiation of a hematopoietic neoplasm.

Keywords: CD141; CD1c; Flow cytometry; JMML; Myeloid dendritic cell.

Publication types

  • Case Reports

MeSH terms

  • Antigens, CD1 / analysis
  • Antigens, Surface / analysis*
  • Biomarkers, Tumor / analysis*
  • Biomarkers, Tumor / genetics
  • Biopsy
  • CD13 Antigens / analysis
  • Cell Differentiation*
  • Child
  • Dendritic Cells / immunology*
  • Dendritic Cells / pathology
  • Flow Cytometry
  • Genetic Predisposition to Disease
  • Glycoproteins / analysis
  • Humans
  • Immunohistochemistry
  • Leukemia, Myelomonocytic, Juvenile / genetics
  • Leukemia, Myelomonocytic, Juvenile / immunology*
  • Leukemia, Myelomonocytic, Juvenile / pathology
  • Leukemia, Myelomonocytic, Juvenile / therapy
  • Male
  • Mutation
  • Phenotype
  • Proto-Oncogene Proteins p21(ras) / genetics
  • Thrombomodulin

Substances

  • Antigens, CD1
  • Antigens, Surface
  • Biomarkers, Tumor
  • CD1C protein, human
  • Glycoproteins
  • KRAS protein, human
  • THBD protein, human
  • Thrombomodulin
  • CD13 Antigens
  • Proto-Oncogene Proteins p21(ras)