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

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.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

Perez-Becker R,Szczepanowski M,Leuschner I,Janka G,Gokel M,Imschweiler T,Völpel S,Niehues T,Klapper W

doi

10.1002/pbc.22756

subject

Has Abstract

pub_date

2011-05-01 00:00:00

pages

859-62

issue

5

eissn

1545-5009

issn

1545-5017

journal_volume

56

pub_type

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