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Association Between Juvenile Myelomonocytic Leukemia, Juvenile Xanthogranulomas and Neurofibromatosis Type 1: Case Report and Review of the Literature
Author(s) -
Paulus Samuel,
Koronowska Sandra,
FölsterHolst Regina
Publication year - 2017
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.13064
Subject(s) - juvenile myelomonocytic leukemia , medicine , juvenile xanthogranuloma , neurofibromatosis , juvenile , presentation (obstetrics) , pediatrics , leukemia , dermatology , pathology , immunology , surgery , genetics , histiocyte , biology , stem cell , haematopoiesis
The 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 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 NF 1 and JXG and provide a possible explanation for this association.