Juvenile xanthogranuloma with hematological dysfunction treated with 2CDA-AraC.
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.
Blouin
Pascale
P
Département de Pédiatrie, Unité d'Hémato Oncologie, CHU Tours, Tours, France. p.blouin@chu-tours.fr
Yvert
Marion
M
Arbion
Flavie
F
Pagnier
Anne
A
Emile
Jean François
JF
Eitenschenck
Laurence
L
Machet
Marie-Christine
MC
Plantaz
Dominique
D
Colombat
Philippe
P
Alla
Corinne Armari
CA
Donadieu
Jean
J
D017338:Cladribine / Q000008:administration & dosage*; D003561:Cytarabine / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005260:Female; D006402:Hematologic Diseases / Q000209:etiology*; D006801:Humans; D007223:Infant; D008297:Male; D014972:Xanthogranuloma, Juvenile / Q000097:blood / Q000188:drug therapy*
D003561:Cytarabine; D017338:Cladribine