Juvenile myelomonocytic leukemia presenting with features of hemophagocytic lymphohistiocytosis in association with neurofibromatosis and juvenile xanthogranulomas

An association exists among neurofibromatosis 1 (NF1), juvenile xanthogranulomas (JXGs), and juvenile myelomonocytic leukemia (JMML). The authors describe a patient with the triple association of JXG, NF1, and JMML initially presenting with features of hemophagocytic lymphohistiocytosis (HLH). An 18...

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Veröffentlicht in:	Journal of pediatric hematology/oncology 2004-09, Vol.26 (9), p.591-595
Hauptverfasser:	Shin, Helen T, Harris, Michael B, Orlow, Seth J
Format:	Artikel
Sprache:	eng
Schlagworte:	Bone Marrow - pathology Fatal Outcome Histiocytosis, Non-Langerhans-Cell - drug therapy Histiocytosis, Non-Langerhans-Cell - pathology Humans Infant Leukemia, Myelomonocytic, Chronic - diagnosis Leukemia, Myelomonocytic, Chronic - drug therapy Male Neurofibromatosis 1 - diagnosis Xanthogranuloma, Juvenile - diagnosis
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container_end_page	595
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container_title	Journal of pediatric hematology/oncology
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creator	Shin, Helen T Harris, Michael B Orlow, Seth J
description	An association exists among neurofibromatosis 1 (NF1), juvenile xanthogranulomas (JXGs), and juvenile myelomonocytic leukemia (JMML). The authors describe a patient with the triple association of JXG, NF1, and JMML initially presenting with features of hemophagocytic lymphohistiocytosis (HLH). An 18-month old boy had multiple cutaneous and gastrointestinal JXG and NF1. At 3 years of age he developed anemia, thrombocytopenia, and hepatosplenomegaly. A bone marrow biopsy revealed features of HLH. Despite chemotherapy, he went on to develop JMML, which proved fatal.
doi_str_mv	10.1097/01.mph.0000135282.39550.99
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subjects	Bone Marrow - pathology Fatal Outcome Histiocytosis, Non-Langerhans-Cell - drug therapy Histiocytosis, Non-Langerhans-Cell - pathology Humans Infant Leukemia, Myelomonocytic, Chronic - diagnosis Leukemia, Myelomonocytic, Chronic - drug therapy Male Neurofibromatosis 1 - diagnosis Xanthogranuloma, Juvenile - diagnosis
title	Juvenile myelomonocytic leukemia presenting with features of hemophagocytic lymphohistiocytosis in association with neurofibromatosis and juvenile xanthogranulomas
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