Neurofibromatosis-Noonan syndrome and acute lymphoblastic leukemia : A report of two cases

• Published in: Journal of pediatric hematology/oncology Vol. 21; no. 2; pp. 158 - 160
• Main Authors: KLOPFENSTEIN, K. J, SOMMER, A, RUYMANN, F. B
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott 01-03-1999
• Subjects: Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Child, Preschool; Chromosomes, Human, Pair 12 - genetics; Chromosomes, Human, Pair 17 - genetics; Genes, Neurofibromatosis 1; Genes, ras; Genetic Predisposition to Disease; Hematologic and hematopoietic diseases; Humans; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Male; Medical sciences; Neurofibromatoses - complications; Neurofibromatoses - genetics; Neurofibromin 1; Noonan Syndrome - complications; Noonan Syndrome - genetics; Phenotype; Precursor Cell Lymphoblastic Leukemia-Lymphoma - drug therapy; Precursor Cell Lymphoblastic Leukemia-Lymphoma - etiology; Proteins - physiology; Remission Induction; Risk; Human; Nervous system diseases; Phacomatosis; Acute; Diseases of the osteoarticular system; Cardiovascular disease; Male; Infant; Malignant hemopathy; Genetic disease; Case study; Concomitant disease; Lymphoproliferative syndrome; Neurofibromatosis; Noonan syndrome; Clinical biology; Cytogenetics; Clinical investigation; Benign neoplasm; Acute lymphocytic leukemia; Osteochondrodysplasia
• ISSN: 1077-4114; 1536-3678
• DOI: 10.1097/00043426-199903000-00014

Two boys with neurofibromatosis-Noonan syndrome in whom acute lymphoblastic leukemia (ALL) developed are described. Both patients demonstrated B-lineage leukemias with normal cytogenetics. They were treated with combination chemotherapy and remain in remission off therapy. Patients with neurofibromatosis-Noonan syndrome may be at increased risk for ALL.