Juvenile Xanthogranuloma, Neurofibromatosis, and Juvenile Chronic Myelogenous Leukemia

Abstract

I read with great interest editorial comments by Riccardi1on possible research implications of article recently published by my colleagues and myself.2However, I was confused by notion of the lack of an excess of inherited cases among patients with triad of NF-1, JXG, and JCML. Our study showed a significant excess of familial neurofibromatosis type 1 (NF-1) among patients with triad compared with patients with NF-1 and juvenile xanthogranuloma (JXG) but without juvenile chronic myelogenous leukemia (JCML) (85% vs 47%,P