[Juvenile myelomonocytic leukaemia, xanthoma, and neurofibromatosis type 1].
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.
Benessahraoui
M
M
Service de dermatologie 1, CHU de Besançon, 2, place Saint-Jacques, 25030 Besançon, France.
Aubin
F
F
Paratte
F
F
Plouvier
E
E
Humbert
P
P
D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use; D015897:Comorbidity; D005260:Female; D006801:Humans; D007223:Infant; D015477:Leukemia, Myelomonocytic, Chronic / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D015122:Mercaptopurine / Q000627:therapeutic use; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000473:pathology; D014972:Xanthogranuloma, Juvenile / Q000150:complications* / Q000473:pathology
D000964:Antimetabolites, Antineoplastic; D015122:Mercaptopurine