Transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma to interdigitating dendritic cell sarcoma: Evidence for transdifferentiation of the lymphoma clone Academic Article uri icon


MeSH Major

  • Cell Transformation, Neoplastic
  • Dendritic Cell Sarcoma, Interdigitating
  • Leukemia, Lymphocytic, Chronic, B-Cell
  • Neoplasms, Multiple Primary


  • Interdigitating dendritic cell sarcoma (IDCS) is a rare tumor derived from interdigitating dendritic cells. Three cases of IDCS associated with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) have been described, but no clonal relationship between the 2 neoplasms was demonstrated. We present a detailed case analysis of a CLL/SLL with metachronous IDCS and demonstrate that these 2 neoplasms are clonally related. The IDCS and CLL cells had trisomy 12 and identical monoclonal immunoglobulin heavy chain gene rearrangements. Analysis of transcription factors with a role in myeloid differentiation demonstrated PU.1 up-regulation and C/EBPalpha down-regulation in IDCS compared with CLL. High-density array comparative genomic hybridization also identified gains in part of chromosome 16q in IDCS. Our study demonstrates for the first time clonal transformation of CLL/SLL into IDCS. This phenomenon may be triggered by alterations in lineage-determining transcription programs, which result in transdifferentiation, coupled with additional oncogenic stimuli caused by chromosomal imbalances.

publication date

  • December 2009



  • Academic Article



  • eng

Digital Object Identifier (DOI)

  • 10.1309/AJCPWQ0I0DGXBMHO

PubMed ID

  • 19926586

Additional Document Info

start page

  • 928

end page

  • 39


  • 132


  • 6