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Juvenile Xanthogranuloma, Neurofibromatosis 1, and Juvenile Chronic Myeloid Leukemia

David H. Gutmann, MD, PhD; James G. Gurney, PhD; Kevin M. Shannon, MD
[+] Author Affiliations

Department of Neurology Washington University School of Medicine 660 S Euclid Ave, Box 8111 St Louis, MO 63110

St Louis

San Francisco, Calif


Arch Dermatol. 1996;132(11):1390-1390. doi:10.1001/archderm.1996.03890350134028
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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

REFERENCES

Shannon KM, O'Connell P, Martin GA, et al.  Loss of the normal NF1 allele from the bone marrow of children with type 1 neurofibromatosis and malignant myeloid disorders . N Engl J Med. 1994;;330:597-601.
Stiller CA, Chessells JM, Fitchett M,  Neurofibromatosis and childhood leukemia/ lymphoma . Br J Cancer. 1994;;70:969-972.
Zvulunov A, Barak Y, Metzker A.  Juvenile xanthogranuloma, neurofibromatosis and juvenile chronic myelogenous leukemia: world statistical analysis . Arch Dermatol. 1995;;131:904-908.
Hennekens CH, Buring JE. Epidemiology in Medicine . Boston, Mass: Little Brown & Co Inc; 1987;:106-108.
Huson SM, Harper PS, Compston DAS.  Von Recklinghausen neurofibromatosis: a clinical and population study in South East Wales . Brain. 1988;;111: 1355-1381.
Kosary CL, Ries LAG, Miller BA, Hankey BF, Harras A, Edwards BK, eds. SEER Cancer Statistics Review, 1973-1992: Tables and Graphs . Bethesda, Md: National Cancer Institute; 1995;: 1-13. NIH publication 96-2789.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

Shannon KM, O'Connell P, Martin GA, et al.  Loss of the normal NF1 allele from the bone marrow of children with type 1 neurofibromatosis and malignant myeloid disorders . N Engl J Med. 1994;;330:597-601.
Stiller CA, Chessells JM, Fitchett M,  Neurofibromatosis and childhood leukemia/ lymphoma . Br J Cancer. 1994;;70:969-972.
Zvulunov A, Barak Y, Metzker A.  Juvenile xanthogranuloma, neurofibromatosis and juvenile chronic myelogenous leukemia: world statistical analysis . Arch Dermatol. 1995;;131:904-908.
Hennekens CH, Buring JE. Epidemiology in Medicine . Boston, Mass: Little Brown & Co Inc; 1987;:106-108.
Huson SM, Harper PS, Compston DAS.  Von Recklinghausen neurofibromatosis: a clinical and population study in South East Wales . Brain. 1988;;111: 1355-1381.
Kosary CL, Ries LAG, Miller BA, Hankey BF, Harras A, Edwards BK, eds. SEER Cancer Statistics Review, 1973-1992: Tables and Graphs . Bethesda, Md: National Cancer Institute; 1995;: 1-13. NIH publication 96-2789.

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