JUVENILE XANTHOGRANULOMA, NEUROFIBROMATOSIS, AND JUVENILE CHRONIC MYELOGENOUS LEUKEMIA - WORLD STATISTICAL-ANALYSIS
JUVENILE XANTHOGRANULOMA, NEUROFIBROMATOSIS, AND JUVENILE CHRONIC MYELOGENOUS LEUKEMIA - WORLD STATISTICAL-ANALYSIS
复制标题
DOI：
10.1001/archderm.131.8.904
复制
发表时间：
1995-08-01
影响因子：
--
通讯作者：
METZKER, A
中科院分区：
文献类型：
--
作者：
ZVULUNOV, A;BARAK, Y;METZKER, A
背景与设计：1型神经纤维瘤病（NF）、青少年慢性髓性白血病（JCML）和青少年黄色肉芽肿（JXG）同时发现已被反复报道。青少年慢性骨髓性白血病在NF患者中更为常见，并可能出现多种皮肤表现，包括JXG。据我们所知，这三个实体之间的关系从未被探索过。本研究的目的是通过对文献的系统回顾来探讨这种关系。我们提出了NF、JCML和JXG之间各种关联的五个示范性病例。结果：85% - 95%的NF和JCML患儿（伴或不伴JXG）有NF家族史，而NF和JXG患儿有NF家族史的比例为47%。观察到的三重关联频率比预期高30至40倍。据估计，患有NF和JXG的儿童患JCML的风险比没有JXG的NF患者高20- 32倍。结论：NF患儿同时发现JCML和JXG代表了一种真正的关联，而不是巧合。在NF婴儿中发现JXG应提醒医生注意可能发展为JCML。
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.