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The prognostic value of serum CA-125 levels both before chemotherapy and after each cycle of one or two courses was assessed in 48 patients with advanced ovarian adenocarcinoma. All patients received a minimum of six courses of either cyclophosphamide and cisplatinum (CP) or cyclophosphamide, doxorubicin and cisplatinum (CAP). Patients with serum CA-125 values below the normal value of 35 U/ml after two courses had a significantly longer median survival (p < 0.0001) and longer disease-free survival (p = 0.007) than did those patients whose CA-125 levels dropped to normal after the third or a later course of chemotherapy. A response in CA-125 levels after the first two courses of chemotherapy may indicate which patients should continue with the current chemotherapy regimen and which patients should be offered salvage therapy.

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.