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Juvenile xanthogranuloma with hematological dysfunction treated with 2CDA‐AraC
Author(s) -
Blouin Pascale,
Yvert Marion,
Arbion Flavie,
Pagnier Anne,
Emile Jean François,
Eitenschenck Laurence,
Machet MarieChristine,
Plantaz Dominique,
Colombat Philippe,
Alla Corinne Armari,
Donadieu Jean
Publication year - 2010
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.22629
Subject(s) - medicine , hepatosplenomegaly , pancytopenia , juvenile xanthogranuloma , surgery , neutropenia , vinblastine , gastroenterology , chemotherapy , disease , pathology , bone marrow , histiocyte
Juvenile xanthogranuloma was diagnosed in two infants aged 8 and 2 months with skin lesions, hepatosplenomegaly, and pancytopenia. Disease control was not achieved with first‐line vinblastine–steroid–VP16 combination therapy. Two courses of 2‐chlorodeoxyadenosine (2CDA) (0.3 mg/kg) and cytosine arabinoside (AraC) (1 g/m 2 ) were then administered for 5 days and were followed, after hematological recovery, by maintenance therapy. Both patients had normal complete blood cell counts and no signs of JXG, respectively, 31 and 24 months after diagnosis. Pediatr Blood Cancer. 2010;55:757–760. © 2010 Wiley‐Liss, Inc.