LOW-GRADE NON-HODGKINS-LYMPHOMA AFTER HIGH-GRADE NON-HODGKINS-LYMPHOMA IN A CHILD WITH ATAXIA TELEANGIECTASIA

Background. Non-Hodgkin lymphomas (NHL) account for approximately 50% of neoplasms in patients with ataxia teleangiectasia (AT). Prognosis i s poor. Published data regarding the treatment of NHL in patients with AT suggested that these children respond poorly to therapy. The autho rs report on an infant with AT with mediastinal high-grade T-NHL who r emained in continuous complete remission after chemotherapy. Diagnosis of AT was established after tumor diagnosis. Methods and Results. The 7-month-old boy was treated according to the acute lymphoblastic leuk emia-Berlin, Frankfurt, Munster 86 protocol. The therapeutic response was prompt, but therapy had to be stopped because of severe side effec ts. Surprisingly, the boy remained in a stable complete remission for 31/2 years. Then tumors in both kidneys occurred and the child died a few months later. Postmortem examination demonstrated large tumors in both kidneys caused by a low-grade malignant lymphoma of B-cell lineag e. Conclusion. Congenital immune deficiency should be ruled out at dia gnosis of rare malignancies in respect to age. NHL in patients with AT can be cured, but poor tolerance to chemotherapy has to be considered . Patients whose disease has been cured may be at high risk for develo pment of a second independent malignancy.