Treatment of the
malignant neoplasms which develop in patients with chromosome fragile syndrome, such as
Bloom's syndrome (BS), requires extremely careful planning of the
chemotherapy regime because of the excessive chemosensitivity of patients with such syndromes. Two siblings with BS developed B cell-type
non-Hodgkin's lymphoma in the third decade of their lives. In both cases a 3-bp homozygous deletion of the BLM gene was detected. Since the
lymphoma of the older brother was nasopharyngeal in origin, he was administrated
radiation therapy as the primary treatment. However, hepatic
metastasis was detected and this was the cause of his death. A 9-bp deletion in exon 7 of the p53 gene was detected in the metastatic
lymphoma. His younger sister developed a
lymphoma of abdominal lymph node in origin. She received a half dose of the drugs used in the
acute lymphoblastic leukemia treatment without radiation, and twenty months after the diagnosis of her
lymphoma she continues to be
in complete remission and free of treatment complications. The p53 gene mutation was not detected in her
lymphoma. These results suggest that
radiation therapy and the radiation dose for the treatment for
lymphoma in patients with BS should be carefully considered.