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.

Cancer Incidence and Survival Among Children and Adolescents in Israel During the Years 1998 to 2007

“…We described families with ataxia telangiectasia and lymphomas in highly inbred Arab families from northern Israel for which a carrier screening program is fully implemented. 30 …”

Chemo- And Radiosensitivity Testing in a Patient With Ataxia Telangiectasia and Hodgkin Disease

“…Based on the above-mentioned data, we think radiotherapy can and should be part of the treatment of Hodgkin disease in AT patients. [19] ABV/COPP(CVPP)# † 22 Secondary leukemia 1995 [19] ABV/COPP# NED 16C 1998 [20] RT † <7 ND 1998 [20] RT † <7 ND 1998 [20] ABVD## † 4 Pulmonary toxicity (nodules) 1998 [20] OPA/ChVPP † 17 ND 1998 [20] ABVD# † 3 Pulmonary toxicity (® brosis) 1998 [20] PE † 28 ND 2000 [29] OPPA † 16 Pneumonia OPPA/ABVD † 6 Pneumonia (CMV) This report RT 900 and OPA## † 6 Progressive disease, pneumonia…”

Hodgkin disease in ataxia‐telangiectasia patients with poor outcomes

“…Eight patients received chemotherapy only (four at 50-75% of standard dose and in four the doses were not documented), two received radiation therapy, two did not have therapy documented in the case report, one received combined modality therapy, and one patient was not treated. In five patients, the Hodgkin disease remitted after reduced-dose chemotherapy (n ¼ 3; 75% of standard dose [10]), after reduced combined modality therapy (n ¼ 1; [14]), and after radiation therapy [9]. Three of these five patients ultimately died of pulmonary complications, one of recurrent Hodgkin disease, and one died of an untreated preleukemic syndrome.…”