Three cases of primary cutaneous lymphoblastic lymphoma: Microarray-based comparative genomic hybridization and gene expression profiling studies with review of literature

Pamela Vezzoli, Francesca Novara, Daniele Fanoni, Daniele Gambini, Ylenia Balice, Luigia Venegoni, Marco Paulli, Carlo Crosti, Emilio Berti

Research output: Contribution to journalArticle

Abstract

Lymphoblastic lymphoma (LBL) is a neoplasm of precursor B- or T-lymphocytes, and primary skin involvement is uncommon. The aim of the study was to review all reported primary cutaneous (PC)-LBLs and to examine three new cases to better characterize this neoplasm. Two of our patients showed a pre-B phenotype (PC-B-LBL) and one a never-reported pre-T phenotype (PC-T-LBL). The patient with PC-T-LBL showed an aggressive course, while those with PC-B-LBL showed a complete remission (CR) after polychemotherapy. Cytogenetic analysis and gene expression profiling (GEP) were performed on one case of PC-B-LBL and on that of PC-T-LBL. A specimen of PC-B-LBL and two specimens (early and late stage) of PC-T-LBL were investigated by microarray-based comparative genomic hybridization (CGH). All specimens revealed trisomy of chromosome 4. PC-T-LBL showed a gain of 1p36.33-p22.1 in the early stage and multiple chromosome gains/losses in the late stage. Our data suggest that trisomy 4 could be detected early in LBL and gain of 1p36.33-p22.1 could be an interesting marker in PC-T-LBL. LBL is an aggressive disease but, only in B-LBL, the cutaneous presentation seems to be a favorable prognostic factor and polychemotherapy is the best therapeutic approach. We suggest that PC-LBL should be included as a provisional clinicopathologic entity in future cutaneous lymphoma classification.

Original languageEnglish
Pages (from-to)1978-1987
Number of pages10
JournalLeukemia and Lymphoma
Volume53
Issue number10
DOIs
Publication statusPublished - Oct 2012

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Keywords

  • Chromosomal aberrations
  • Gene expression profiling (GEP)
  • Microarray comparative genomic hybridization (CGH)
  • Precursor T-cell lymphoblastic lymphoma
  • Primary cutaneous lymphoblastic lymphoma
  • Trisomy 4

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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