title: Acute lymphoblastic leukemia in Ehlers-Danlos syndrome.
abstract: We report a girl with type II Ehlers-Danlos syndrome who developed acute lymphoblastic leukemia. At the age of 5 years, she was referred to our hospital with mild hyperelasticity of skin and mild joint hypermobility, 'cigarette paper' wrinkled scarring of the forehead, and moderate ecchymoses. She was diagnosed with type II Ehlers-Danlos syndrome (mitis type). Ten months after referral, she developed acute lymphoblastic leukemia (FAB classification; L1). Her leukemia cells expressed both CD 10 and HLA-DR antigens. We treated her with L-asparaginase, prednisolone, vincristine, doxorubicin, methotrexate, and cytosine arabinoside and she has remained disease-free for 6 years since the first remission. To our knowledge, this is the first report of Ehlers-Danlos syndrome in a patient who developed acute lymphoblastic leukemia.
authors.affiliation: Department of Pediatrics, Nippon Medical School, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Asano
authors.forename: M
authors.initials: M
authors.lastname: Yamamoto
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000008:administration & dosage; D002675:Child, Preschool; D003561:Cytarabine / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D008727:Methotrexate / Q000008:administration & dosage; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications* / Q000188:drug therapy; D011239:Prednisolone / Q000008:administration & dosage; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D003561:Cytarabine; D014750:Vincristine; D004317:Doxorubicin; D011239:Prednisolone; D001215:Asparaginase; D008727:Methotrexate