TREATMENT OF LYMPHOID MALIGNANCIES IN PATIENTS WITH ATAXIA-TELANGIECTASIA

Background, Patients with ataxia-telangiectasia (A-T) are at an increa sed risk for developing lymphoid malignancies, yet the appropriate the rapy remains unknown. Radiation therapy at conventional doses results in destruction of normal tissue, which has suggested that full-dose ch emotherapy might result in unacceptable toxicity in A-T patients with cancer. Procedure, The medical records of 412 A-T patients were review ed to identify those patients who developed lymphoid malignancies and to analyze the type and duration of therapy, events during therapy, an d off-therapy follow-up. Results. Of 74 A-T patients with lymphoid mal ignancies, 32 patients received chemotherapy. The 21 patients treated with standard chemotherapy had a significantly better median survival (9 months, range, 1-162+ months vs. 5 months, range, 0.5-28 months) (P = 0.03) and complete remission rate (76% vs. 9%) (P = 0.001) than the 11 treated with reduced dose chemotherapy. Three of the 21 lull-dose c hemotherapy patients required dose reductions because of neutropenia. Seven of the 14 patients exposed to 1,200 mg/m(2) or greater of cyclop hosphamide developed hemorrhagic cystitis. All three patients exposed to bleomycin developed pulmonary disease which was fatal in two. Of th e 16 standard-dose chemotherapy patients who achieved a complete remis sion, two remain disease-free, five have died of recurrent disease, an d five died of pulmonary disorders and four of other causes while in r emission. Conclusions, Standard-dose chemotherapy should be given to e ach A-T patient with a lymphoid malignancy unless additional physical or emotional problems make it unlikely that the patient will benefit. Morbidity and mortality may be reduced by prophylaxis against hemorrha gic cystitis and early detection and treatment of pulmonary disorders. (C) 1998 Wiley-Liss, Inc.