Association between juvenile myelomonocytic leukemia, juvenile xanthogranulomas and neurofibromatosis type 1: case report and review of the literature
S Paulus, S Koronowska… - Pediatric dermatology, 2017 - Wiley Online Library
S Paulus, S Koronowska, R Fölster‐Holst
Pediatric dermatology, 2017•Wiley Online LibraryThe occurrence of juvenile myelomonocytic leukemia (JMML), juvenile xanthogranuloma
(JXG), and neurofibromatosis type 1 (NF 1) together is relatively rare. Approximately only 20
cases have been reported in the literature. It is debated whether children with NF 1 and JXG
are at higher risk of developing JMML than children with NF 1 alone. We present the case of
a boy primarily diagnosed with NF 1 with coexisting JXG who developed JMML at the age of
22 months. The clinical course from initial presentation to final diagnosis is detailed and the …
(JXG), and neurofibromatosis type 1 (NF 1) together is relatively rare. Approximately only 20
cases have been reported in the literature. It is debated whether children with NF 1 and JXG
are at higher risk of developing JMML than children with NF 1 alone. We present the case of
a boy primarily diagnosed with NF 1 with coexisting JXG who developed JMML at the age of
22 months. The clinical course from initial presentation to final diagnosis is detailed and the …
Abstract
The occurrence of juvenile myelomonocytic leukemia (JMML), juvenile xanthogranuloma (JXG), and neurofibromatosis type 1 (NF1) together is relatively rare. Approximately only 20 cases have been reported in the literature. It is debated whether children with NF1 and JXG are at higher risk of developing JMML than children with NF1 alone. We present the case of a boy primarily diagnosed with NF1 with coexisting JXG who developed JMML at the age of 22 months. The clinical course from initial presentation to final diagnosis is detailed and the genetic features and hematologic characteristics are discussed. We report this case to underscore the importance of close monitoring of blood count and strict clinical follow‐up in children presenting with concurrent NF1 and JXG and provide a possible explanation for this association.
Wiley Online Library