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  • Title: [Juvenile myelomonocytic leukaemia, xanthoma, and neurofibromatosis type 1].
    Author: Benessahraoui M, Aubin F, Paratte F, Plouvier E, Humbert P.
    Journal: Arch Pediatr; 2003 Oct; 10(10):891-4. PubMed ID: 14550978.
    Abstract:
    The triple association of leukemia, xanthogranulomas, and type 1 neurofibromatosis was first described in 1958. Most leukemias were juvenile myelomonocytic leukemias (JMML), usually called juvenile chronic myelogenous leukemia. We describe a 22-month-old female child with neurofibromatosis 1, xanthomagranulomas, and a JMML. Her mother and her brother also had cutaneous café-au-lait spots. Our patient was treated with mercaptopurine and improved. However, 9 months later she experienced a blastic transformation. The presence of xanthomagranulomas and NF1 in a young child should alert to a possible development of JMML, especially in patients with a family history of NF1.
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