SummaryHigh cure rates are possible in children with localized mature B-cell lymphoma (B NHL) using a variety of chemotherapeutic strategies. To reduce late sequelae, the duration and intensity of chemotherapy has been progressively reduced. The Lymphome Malins de Burkitt (LMB) 89 study reported long-term survival in almost all children with localized resected disease treated with two courses of COPAD (cyclophosphamide, vincristine, prednisolone and doxorubicin). This study was designed to confirm the effectiveness of this approach in a larger number of patients in a multinational co-operative study. The patient cohort was part of an international study (French-American-British LMB 96), which included all disease stages and involved three national groups. Patients in this part of the study had resected stage I or completely resected abdominal stage II disease. Following surgery, two courses of COPAD were given, without intrathecal (IT) chemotherapy. One hundred and thirty-two children were evaluable. Two of 264 (0AE9%) courses were associated with grade IV toxicity (one stomatitis and one infection). With a median follow up of 50AE5 months, the 4 year event-free survival is 98AE3% and overall survival is 99AE2%. Children with resected localized B-NHL can be cured with minimal toxicity following two courses of low intensity treatment without IT chemotherapy.

No abstract

A B S T R A C T Flaujeac trait plasma resembled Hageman trait or Fletcher trait, in that the intrinsic coagulation pathway, plasma fibrinolytic pathway, kinin-forming system, permeability factor of dilution (PF/dil) phenomenon were abnormal. The defect in each assay was reconstituted by a factor separable from Hageman factor or Fletcher factor. This substance was an a-globulin with an approximate mol wt of 170,000. Flaujeac plasma did not release a kinin upon incubation with kallikrein and was deficient in total kininogen antigen. Antiserum to kininogen inhibited the activity of the factor in solution. Flaujeac factor was identified as a kininogen of high molecular weight (HMW-kininogen).The mean total kininogen antigen in four children of the proposita was 51% (range 34-62%) of normal. A functional coagulation assay of HMW-kininogen in the children was 34% (range 23-55%). The results were consistent with autosomal recessive inheritance.The plasma pathways of intrinsic coagulation, fibrinolysis, kinin formation, and PF/dil generation are dependent upon HMW-kininogen. We believe this is the first demonstration of biological function for a kininogen apart from its role as a substrate for kallikreins.

The hallmarks of diagnosis of medullary breast cancer (MedBC) used by the authors since 1977 have been that the tumor is well circumscribed, has syncytial architecture in greater than 75% of its surface, contains diffuse inflammatory infiltrate, has atypical nuclei, and forms no glandular pattern. In order to assess the clinical utility of these criteria, we studied a series of 95 previously untreated, surgically operable patients with breast carcinoma at the Institut Gustave-Roussy (IGR) between 1960 and 1979. A diagnosis of MedBC was initially made for these patients or suspected based on abundant inflammatory stroma observed in a histologic evaluation. Using these criteria, 26 cases were identified as typical medullary carcinoma (TMC), 23 cases as atypical medullary carcinoma (AMC), and 46 cases as nonmedullary carcinoma (NMC). The 26 cases of TMC represent a very small fraction of the total infiltrating operable breast carcinomas diagnosed at IGR during the same time period. The prognosis for these 26 patients was much more favorable than for the other groups. They had a 10-year disease-free survival of 92% compared with 53% for the AMC group and 51% for the NMC group. Neither distant metastasis nor secondary primaries of the same histology were seen. Therefore, it is possible with the use of strict histologic criteria to distinguish a group of patients with a much more favorable prognosis. This histologic diagnosis alone renders a most favorable prognosis for the patient even if other factors such as large tumor size and lymph node involvement are present and, by inference, the only therapy needed is the removal of all tumor. In contrast, atypical forms have a prognosis no different from other atypical types of breast carcinomas without inflammatory stroma, and adjuvant therapy appears to be justified if other factors warrant it.

No abstract