title: Ataxia telangiectasia and lymphoma: an indication for individualized chemotherapy dosing--report of treatment in a highly inbred Arab family.
abstract: Ten of 18 children in a highly inbred Arab kindred suffered from either ataxia telangiectasia (AT) or a variant syndrome consisting of ataxia, microcephaly, and congenital cataract (AMC). Four of the nine afflicted children were treated in our unit when they developed lymphomas (both Hodgkin's and non-Hodgkin's including Burkitt's). They were given chemotherapy (either standard COMP or low-dose ABV/CVPP). The children with non-Hodgkin's lymphomas died of sepsis after receiving full-dose COMP. Low-dose ABV/CVPP brought about a 20-month remission in one child with nodular sclerosing Hodgkin's lymphoma and both AT and AMC, but she developed a preleukemic syndrome and her parents refused further treatment; she too died. A fourth child, also with nodular sclerosing Hodgkin's lymphoma, is currently in complete remission after ABV/CVPP. Treatment of lymphomas in patients with AT is extraordinarily difficult and has potential side effects so grave as to necessitate careful monitoring and individualized protocols.
authors.affiliation: Pediatric Oncology Unit, Rambam Medical Center, Haifa, Israel.
authors.forename: M
authors.initials: M
authors.lastname: Weyl Ben Arush
authors.forename: J
authors.initials: J
authors.lastname: Rosenthal
authors.forename: J
authors.initials: J
authors.lastname: Dale
authors.forename: Y
authors.initials: Y
authors.lastname: Horovitch
authors.forename: G
authors.initials: G
authors.lastname: Herzl
authors.forename: J
authors.initials: J
authors.lastname: Ben Arie
authors.forename: Y
authors.initials: Y
authors.lastname: Ziv
authors.forename: Y
authors.initials: Y
authors.lastname: Shiloh
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001260:Ataxia Telangiectasia / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008223:Lymphoma / Q000150:complications / Q000188:drug therapy*; D010375:Pedigree; D012529:Saudi Arabia; D013577:Syndrome