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August 1995

Juvenile Xanthogranuloma, Neurofibromatosis, and Juvenile Chronic Myelogenous LeukemiaWorld Statistical Analysis

Author Affiliations

From the Dermatology Unit, Children's Medical Center of Israel, Beilinson Campus, Petah Tiqwa, and Tel Aviv (Israel) University Sackler Medical School (Drs Zvuluno and Metzker), and the Department of Pediatrics "A," Kaplan Hospital, Rehovot and Hadassah Hebrew University Medical School, Jerusalem, Israel (Dr Barak). Dr Zvuluno is now with the Department of Dermatology, Soroka Medical Center, Beer-Sheva, Israel.

Arch Dermatol. 1995;131(8):904-908. doi:10.1001/archderm.1995.01690200040007

Background and Design:  The concurrent finding of neurofibromatosis type 1 (NF), juvenile chronic myelogenous leukemia (JCML), and juvenile xanthogranuloma (JXG) has been repeatedly reported. Juvenile chronic myelogenous leukemia has been found more frequently in patients with NF and may present with various cutaneous manifestations, including JXG. To our knowledge, the relationship among these three entities has never been explored. The purpose of the present study is to explore this relationship by using a systematic review of the literature. We present five demonstrative cases of various associations among NF, JCML, and JXG.

Results:  A family history of NF was found in 85% to 95% of children with NF and JCML (with or without JXG), as compared with that found in 47% of children with NF and JXG. The observed frequency of the triple association is 30-fold to 40-fold higher than that expected. It is estimated that children with NF and JXG have a 20-fold to 32-fold higher risk for JCML than do patients with NF who do not have JXG.

Conclusions:  A concomitant finding of JCML and JXG in children with NF represents a true association, rather than a coincidence. A finding of JXG in an infant with NF should alert a physician to a possible development of JCML.(Arch Dermatol. 1995;131:904-908)