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This study was designed to determine the prognostic significance of multidrug resistance, mediated by P-glycoprotein (Pgp) expression, in Ewing sarcoma. The clinical and laboratory features, treatment protocol, and outcome of 75 patients with Ewing sarcoma or peripheral neuroectodermal tumor treated between 1972 and 1997 were reviewed. Pgp expression was tested with the monoclonal antibody JSB-1. Thirty-four (64%) of the 53 tissue samples from untreated patients stained positive for Pgp. Progression-free and overall survival were 44 and 59%, respectively, in patients with negative findings, and 28 and 41% in those with positive findings; neither difference was significant. Of the 12 relapsed patients, 6 (50%) expressed more Pgp after chemotherapy than at diagnosis and 4 (33%) expressed less. Within these subgroups, 5 out of 6 and 3 out of 4 died from the disease. No correlation was found between Pgp and known prognostic factors of Ewing tumors. Pgp expression is probably an intrinsic factor of Ewing tumors but has no correlation to prognosis. [ABSTRACT FROM AUTHOR]

Sixty-four adult survivors of childhood cancer, recruited via Israel's largest pediatric cancer treatment center, participated in a multi-dimensional assessment of long-term adjustment and quality of life in the domains of educational achievement, employment status, military service, family status, health, and psychological well-being. Subjects had been diagnosed with cancer prior to age 18, were three years or more off therapy with no evidence of disease, and over 18 years old at the time of the study. Data from structured interviews were compared to responses on similar items from a control group with no history of serious illness during childhood, matched for age, sex, and parental education levels. Results indicated an overall pattern of integration into the social mainstream, with similar objective levels of achievement for survivors and controls for most measures of education, employment, significant relationships, and psychological well-being. Results also indicated certain areas of disadvantage, such as military recruitment difficulties, lower income levels, and higher rates of workplace rejection. Significantly, almost half of the survivor sample reported subjective feelings that their illness experience had impaired their achievement in several domains. Quality of life is considered an important outcome parameter in terms of clinical decision making as well as in guiding preventive and supportive intervention efforts. [ABSTRACT FROM AUTHOR]

Congenital dyserythropoietic anemia (CDA) type I is a rare inherited bone marrow disorder characterized by moderate to severe macrocytic anemia with pathognomonic cytopathology of nucleated red blood cells. Previous studies have suggested that serum erythropoietin levels in affected patients are lower than expected for the degree of anemia. An earlier study demonstrated a substantial increase in the number of CFU-E in CDA type I pattern on addition of exogenous erythropoietin. The present study reports on the response to recombinant human erythropoietin in 8 patients with CDA type I. Eighteen weeks of treatment, starting at 300 IU/kg twice a week and gradually increasing to 500 IU/kg three times a week, did not have a substantial effect on the mean hemoglobin value. These results indicate that recombinant human erythropoietin (rHuEpo) is not beneficial to patients with CDA type I and that the relatively low levels of serum erythropoietin probably play no major role in the pathogenesis of the disease. [ABSTRACT FROM AUTHOR]