Ataxia telangiectasia and lymphoma: An indication for individualized chemotherapy dosing - report of treatment in a highly inbred arab family

M. Weyl Ben Arush*, J. Rosenthal, J. Dale, Y. Horovitch, G. Herzl, J. Ben Arie, Y. Ziv, Y. Shiloh

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)163-169
Number of pages7
JournalPediatric Hematology and Oncology
Volume12
Issue number2
DOIs
StatePublished - 1995

Keywords

  • Ataxia telangiectasia
  • Chemotherapy
  • Lymphoma

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