The association between juvenile xanthogranulomas in neurofibromatosis type 1 patients and the development of leukaemia: A systematic review.

This systematic review examines the relationship between neurofibromatosis type 1 (NF1), juvenile xanthogranulomas (JXG), and juvenile myelomonocytic leukemia (JMML). The study reveals that JMML is more prevalent in NF1 patients with JXG compared to those without. Patients with NF1, JXG, and JMML tend to be younger at diagnosis, have earlier detection of JXG, and have larger JXG compared to other groups. The study also finds that leukaemic cells in patients with JMML show evidence of biallelic inactivation of NF1. The article suggests that increased monitoring for JMML may be necessary in NF1 patients with certain risk factors, such as a positive family history of NF1, male gender, and early onset and larger size of JXG. [Extracted from the article]