title: Fatal eosinophilic heart disease in a child with neurofibromatosis-1 complicated by acute lymphoblastic leukemia.
abstract: We present a pediatric case of neurofibromatosis-1 (NF-1) complicated by acute lymphoblastic leukemia and hypereosinophilia, which caused multiple end-organ damage. Although clinical symptoms such as fever and coughing were noted only 1 week before admission, the condition deteriorated rapidly with a fatal outcome prior to antileukemic therapy. A postmortem examination demonstrated extensive endomyocardial fibrosis with thrombotic occlusion and recanalization of the coronary arteries. Leukemic cell infiltration was not seen in the cardiac tissue. When eosinophilia is diagnosed in patients with NF-1, eosinophilic end-organ damage, particularly cardiac involvement, in addition to hematological malignancies, should be screened for in order to start medical treatment at the early stage of the disease.
authors.affiliation: Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, 305-8575, Japan. hhorigom@md.tsukuba.ac.jp
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Horigome
authors.forename: Ryo
authors.initials: R
authors.lastname: Sumazaki
authors.forename: Nobuaki
authors.initials: N
authors.lastname: Iwasaki
authors.forename: Natsuki
authors.initials: N
authors.lastname: Imoto
authors.forename: Hideyo
authors.initials: H
authors.lastname: Kinugasa
authors.forename: Makoto
authors.initials: M
authors.lastname: Saito
authors.forename: Akira
authors.initials: A
authors.lastname: Matsui
mesh_terms: D002675:Child, Preschool; D017809:Fatal Outcome; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications*; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications*