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An aggressive systemic juvenile xanthogranuloma clonally related to a preceding T‐cell acute lymphoblastic leukemia |
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| Authors: | Ruy Perez‐Becker MD Monika Szczepanowski PhD Ivo Leuschner MD Gritta Janka MD PhD Michael Gokel MD Thomas Imschweiler MD Sigrid Völpel MD Tim Niehues MD Wolfram Klapper MD |
| Institution: | 1. HELIOS Klinikum Krefeld, Zentrum für Kinder‐ und Jugendmedizin, Lutherplatz 40, 47805 Krefeld, Germany;2. Universit?tsklinikum Schleswig‐Holstein, Campus Kiel, Institut für Pathologie, Sektion H?matopathologie und Lymphknotenregister Kiel, Haus 14, Arnold‐Heller‐Strasse 3, 24105 Kiel, Germany;3. Universit?tsklinikum Hamburg‐Eppendorf, Zentrum für Frauen‐, Kinder und Jugendmedizin, Martinstrasse 52, 20246 Hamburg, Germany;4. HELIOS Klinikum Krefeld, Institut für Pathologie, Lutherplatz 40, 47805 Krefeld, Germany |
| 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. Pediatr Blood Cancer 2011;56:859–862. © 2011 Wiley‐Liss, Inc. |
| Keywords: | histiocytosis juvenile xanthogranuloma T‐cell acute lymphoblastic leukemia |