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  Vol. 132 No. 11, November 1996 TABLE OF CONTENTS
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Juvenile Xanthogranuloma, Neurofibromatosis 1, and Juvenile Chronic Myeloid Leukemia

David H. Gutmann, MD, PhD
Department of Neurology Washington University School of Medicine 660 S Euclid Ave, Box 8111 St Louis, MO 63110

James G. Gurney, PhD
St Louis

Kevin M. Shannon, MD
San Francisco, Calif

Arch Dermatol. 1996;132(11):1390.

Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

Neurofibromatosis 1 (NF1) is a common autosomal dominant disorder in which affected children have an approximate 200-fold greater risk of developing juvenile chronic myeloid leukemia (JCML) compared with children without NF1.1,2 A recent article in the ARCHIVES3 presented the results of a statistical analysis from a compilation of case reports suggesting that children with NF1 have an even higher risk of JCML (>20-fold) if they concurrently have juvenile xanthogranuloma (JXG). Although case reports are useful for generating hypotheses, because case reports are not drawn from defined populations and do not include representative comparison groups they cannot be used to test for the presence of valid statistical associations.4 The methodological limitations of such a case report analysis coupled with an apparent overestimate of the disease probabilities used by Zvulunov et al3 raise several concerns. They estimate the frequency of NF1 to be between 1 per 2500 and . . . [Full Text PDF of this Article]



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