Primary cutaneous CD30 positive T-cell lymphoproliferative disorders with aberrant expression of PAX5: Report of three cases

Masahiro Hagiwara, Akihiro Tomita, Katsuyoshi Takata, Yoshie Shimoyama, Tadashi Yoshino, Yasushi Tomita, Shigeo Nakamura

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Accurate diagnosis of lymphoma includes the assessment of lineage-specific markers. Hematopoietic and lymphoid tissues express PAX5 exclusively in pro-B-cell to mature B-cell stages. However, some mature PAX5+ T-cell lymphomas have been reported. We report three cases of primary cutaneous CD30+ T-cell lymphoproliferative disorders (LPDs) with PAX5 expression: one cutaneous anaplastic large cell lymphoma (ALCL) and two cases of lymphomatoid papulosis (LyP). The three patients were 26years old and female, 75years old and female, and 65years old and male. In all cases, Hodgkin's and Reed-Sternberg-like large lymphoid cells were present, positive for CD30, fascin, and PAX5, and negative for CD3, CD4, CD8, CD20, CD45RO, CD56, cytotoxic markers, and Epstein-Barr virus. The ALCL was accompanied by lymphadenopathy; the patient died of progressive disease 5months after diagnosis. The LyP cases were localized in the skin with spontaneous regression. One case was diagnosed during pregnancy, transformed to ALCL, and ended in death 32months after diagnosis despite multi-agent chemotherapy. This study is the first to address the clinical significance of PAX5+ primary cutaneous CD30+ T-cell LPDs. These cases were distinct regarding PAX5 expression and a relatively aggressive clinical course versus conventional primary cutaneous CD30+ T-cell LPDs.

Original languageEnglish
Pages (from-to)264-270
Number of pages7
JournalPathology International
Volume62
Issue number4
DOIs
Publication statusPublished - Apr 2012

Keywords

  • Cutaneous anaplastic large cell lymphoma
  • Lymphomatoid papulosis
  • PAX5
  • Pregnancy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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