Precocious puberty following BMT.
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Biomedical subjects
Publications and source records attributed to L Rebuffi.
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PTC, a mixture of oligopeptides of m-L-sarcholysin, acting primarily as an alkylating agent, was utilized as initial therapy following diagnosis in 80 children with nonlocalized neuroblastoma. Of the 67 evaluable patients (21 Stage III, 41 Stage IV and five Stage IV-S), 51 had measurable lesions allowing to evaluate PTC activity; objective tumor responses to the drug were recorded in 45 of these 51 cases (88.2%); 5/5 Stage III, 37/41 Stage IV, 3/5 Stage IV-S. Complete responses were obtained in seven patients (13.7%), partial responses in 32 (62.7%), objective improvement in six (11.8%). Four patients (7.8%) had either no tumor change, or tumor progression. There have been two early drug-related deaths (3.9%). Stage III and IV patients responding to PTC were then treated by irradiation + VCR, followed by cycles of a combination of ADriamycin, vincristine, and cyclophosphamide. Stage IV-S patients received no further therapy. Thirteen of 21 Stage III (61.9%), five of 41 Stage IV (12.2%) and four of five Stage IV-S (80%) are presently alive from 19-48 months (median, 27 months). PTC is an effective agent in advanced neuroblastoma. However, the results of this report do not indicate that its addition to a "standard" treatment, at least in the schedule adopted in this protocol, has improved the final outcome of children with nonlocalized disease.
A 14-month-old child with congenital hypoplastic anemia, in temporary remission after steroid therapy, developed an acute megakarioblastic leukemia. After complete remission due to immunosuppressive treatment, the leukemia rapidly relapsed, and the child died at 23 months of age. The rapid development of leukemia puts some doubt on the initial diagnosis.
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We present a patient with a Wilms' Tumor recurring after 7 years from the primitive surgical excision. After the relapse, surgery and conventional chemotherapy (with Actinomycin-D, Cyclophosphamide and Vincristine) permitted to achieve a second complete remission. Now the patient is alive without evidence of disease after 4 years from the relapse. The non-complete surgical excision of the tumor and the particular biological activity of the residual cells could explain the late relapse.
The authors discuss the CT findings of the late brain damage by the radiochemotherapic treatment for the prophylaxis of meningeal leukemia in 50 patients, 1 divided by 15 years old. The more frequent CT findings are: enlargement of the subaracnoid spaces; ventricular dilatation; hypodensity of cerebral parenchima; calcifications. These findings correspond to two clinical and anatomo-pathological conditions: subacute leucoencephalopathy and mineralizing microangiopathy. A greater damage on central nervous system was demonstrated in younger children (5 years old) at the time of prophylaxis of meningeal leukemia, that were irradiated with NSD = 950 ret.