Expression of CD5 on hematogones in a 7-year-old girl with Shwachman-Diamond syndrome

Pediatr Dev Pathol. 2001 Sep-Oct;4(5):505-11. doi: 10.1007/s10024001-0083-5.

Abstract

We report increased numbers of hematogones in a 7-year-old girl with pancytopenia due to Shwachman-Diamond syndrome. Her hematogones expressed the T-cell marker CD5 as well as CD19, CD10, and CD20, and terminal deoxynucleotidyl transferase and HLA-DR. These findings suggest that hematogones are precursors of both CD5-positive B cells and CD5-negative B cells. Thus CD5-positive B cells in bone marrow may be derived from bone marrow stem cells, and not from the residual fetal B cells of yolk sac/liver origin. The finding of CD5 expression on hematogones also raises the possibility that neoplastic B cells of chronic lymphocytic leukemia, which characteristically co-express CD5 and CD19, may be derived from CD5-positive B-cell precursors in bone marrow and not from mature B cells in lymph nodes.

Publication types

  • Case Reports

MeSH terms

  • B-Lymphocytes / metabolism
  • B-Lymphocytes / pathology*
  • Bone Marrow Cells / metabolism
  • Bone Marrow Cells / pathology
  • CD5 Antigens / biosynthesis*
  • Child
  • DNA Nucleotidylexotransferase / biosynthesis
  • Exocrine Pancreatic Insufficiency / diagnostic imaging
  • Exocrine Pancreatic Insufficiency / metabolism
  • Exocrine Pancreatic Insufficiency / pathology*
  • Female
  • Flow Cytometry
  • Growth Disorders
  • HLA-DR Antigens / biosynthesis
  • Hematopoietic Stem Cells / metabolism
  • Hematopoietic Stem Cells / pathology*
  • Humans
  • Immunoenzyme Techniques
  • Immunophenotyping
  • In Situ Hybridization, Fluorescence
  • Leukemia, Lymphocytic, Chronic, B-Cell / etiology
  • Leukemia, Lymphocytic, Chronic, B-Cell / metabolism
  • Leukemia, Lymphocytic, Chronic, B-Cell / pathology
  • Pancreas / diagnostic imaging
  • Pancreas / pathology
  • Syndrome
  • Tomography, X-Ray Computed

Substances

  • CD5 Antigens
  • HLA-DR Antigens
  • DNA Nucleotidylexotransferase