An aggressive systemic juvenile xanthogranuloma clonally related to a preceding T-cell acute lymphoblastic leukemia.
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.
Perez-Becker
Ruy
R
HELIOS Klinikum Krefeld, Zentrum für Kinder- und Jugendmedizin, Lutherplatz 40, 47805 Krefeld, Germany.
Szczepanowski
Monika
M
Leuschner
Ivo
I
Janka
Gritta
G
Gokel
Michael
M
Imschweiler
Thomas
T
Völpel
Sigrid
S
Niehues
Tim
T
Klapper
Wolfram
W
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects*; D001483:Base Sequence; D002675:Child, Preschool; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D007107:Immune System; D008221:Lymphoid Tissue / Q000473:pathology; D008969:Molecular Sequence Data; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications / Q000188:drug therapy*; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics*; D014972:Xanthogranuloma, Juvenile / Q000139:chemically induced* / Q000235:genetics* / Q000473:pathology
D016692:Receptors, Antigen, T-Cell, gamma-delta