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An aggressive systemic juvenile xanthogranuloma clonally related to a preceding T‐cell acute lymphoblastic leukemia
Author(s) -
PerezBecker Ruy,
Szczepanowski Monika,
Leuschner Ivo,
Janka Gritta,
Gokel Michael,
Imschweiler Thomas,
Völpel Sigrid,
Niehues Tim,
Klapper Wolfram
Publication year - 2011
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22756
Subject(s) - juvenile xanthogranuloma , medicine , histiocyte , pathology , t cell receptor , lymph node , gene rearrangement , leukemia , immunology , t cell , gene , biology , genetics , immune system
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. Pediatr Blood Cancer 2011;56:859–862. © 2011 Wiley‐Liss, Inc.