An aggressive systemic juvenile xanthogranuloma clonally related to a preceding T-cell acute lymphoblastic leukemia

Pediatr Blood Cancer. 2011 May;56(5):859-62. doi: 10.1002/pbc.22756. Epub 2010 Sep 16.

Abstract

Juvenile xanthogranuloma (JXG) is a disorder of disputed origin thought to be related to the dermal/interstitial macrophage. A 5-year-old female presented with an aggressive systemic JXG that developed 5 months after the diagnosis of T-cell acute lymphoblastic leukemia (T-ALL). Examination of the T-cell receptor gamma (TCR-γ) rearrangement in T-ALL blasts, JXG infiltrated lymph node biopsies and micro-dissected JXG histiocytes revealed an identical bi-allelic TCR-γ rearrangement in all samples, thus providing evidence for a clonal relationship between T-ALL and JXG in this case.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / adverse effects*
  • Base Sequence
  • Child, Preschool
  • Female
  • Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
  • Histiocytes / pathology
  • Humans
  • Immune System
  • Lymphoid Tissue / pathology
  • Molecular Sequence Data
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / complications
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / drug therapy*
  • Receptors, Antigen, T-Cell, gamma-delta / genetics*
  • Xanthogranuloma, Juvenile / chemically induced*
  • Xanthogranuloma, Juvenile / genetics*
  • Xanthogranuloma, Juvenile / pathology

Substances

  • Receptors, Antigen, T-Cell, gamma-delta