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Outpatient adherence to oral medication regimens was evaluated in 50 adolescents and young adults with cancer: 21 patients with acute lymphoblastic leukemia or Hodgkin disease who were to take prednisone as a component of their chemotherapy regimen, and 29 patients with Hodgkin disease whose chemotherapy had been discontinued and who were to take penicillin for postsplenectomy prophylaxis. Of the 21 adolescent patients with acute lymphoblastic leukemia or Hodgkin disease who were to take prednisone as outpatients, 11 patients were found to be nonadherent to treatment (52%). Among the 29 adolescents for whom penicillin was prescribed for postsplenectomy prophylaxis, nonadherence was detected in 14 patients (48%). If a method is available, laboratory assessment to determine adherence should be performed in adolescents and young adults receiving long-term therapy, particularly if one wishes to draw valid conclusions regarding efficacy of various treatment protocols.

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The focus of our study was to identify specific demographic, treatment, and psychologic factors that may differentiate patients who complied with cancer therapy from those who did not. In a companion study, bioassay techniques revealed frequent nonadherence to oral medication regimens among 50 adolescents and young adults with acute lymphoblastic leukemia or Hodgkin disease. In this sample, compliers and noncompliers did not differ with respect to either demographic variables (age, gender) or treatment variables (treatment duration, continuity, complexity, or parental involvement). Psychologic variables were assessed in 34 patients. Patients who adhered poorly to the therapeutic regimen had significantly less developed concepts of the illness (specifically, concepts of causality and prognosis), less perceived vulnerability (especially as related to subclinical disease), higher levels of denial as a psychologic defense, and less cohesive future orientation. The perceived health locus of control was not a significant factor. The results suggest that patients construct their own subjective view of the illness and its treatment, which then has implications for adherence to medication regimens.

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A 14-year-old girl presented with a large abdominal mass, thought to be an ovarian tumor on the basis of clinical and sonographic findings. MRI correctly suggested a primary colonic neoplasm. At operation, ovarian involvement was found to be secondary to metastatic signet ring cell adenocarcinoma of the colon, an extremely rare malignancy in this age group. Low index of suspicion for this tumor in children and adolescents results in advanced disease at diagnosis and poor prognosis. Early imaging with MRI is recommended when the nature of pelvic disease in children remains obscure.

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Six children with juvenile chronic myelocytic leukemia (JCML) with adverse prognostic features were treated with intensive combination chemotherapy similar to that utilized in patients with acute nonlymphocytic leukemia (ANLL). Despite obtaining hematologic remissions after induction therapy, clinical findings of extramedullary disease persisted. The use of intensive post-induction chemotherapy did not erradicate persistent extramedullary disease, and all patients developed hematologic relapse and progressive disease at a median of 8 months. The median survival of the treated patients was 15 months. The use of intensive ANLL therapy in poor prognosis JCML does not improve the survival rates reported with less intensive regimens but does have value in producing hematologic remissions that may be useful in preparing patients for bone marrow transplant.

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We measured dehydroepiandrosterone sulfate (DHEA-S) levels in children before and after high dose prednisone therapy. In older children (postadrenarchal), there was a 70% decrease in DHEA-S levels after 1 week. However, even after a month of therapy, DHEA-S was detectable in serum. In contrast, in younger (preadrenarchal) children, the low initial DHEA-S levels were not decreased by prednisone therapy. These findings suggest that there are two distinct regulatory pathways leading to DHEA-S, one of which is independent of ACTH.

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A 5 1/2-year-old child with Bloom's syndrome developed acute lymphocytic leukemia (ALL). Bloom's syndrome is associated with chromosomal aberrations, and affected individuals have an increased incidence of leukemia and solid tumors. The skin on our patient had adjacent areas of decreased and increased pigmentation similar to the "twin-spots" seen in Drosophila. "Twin-spots" are the manifestation of somatic cell DNA recombination and provide evidence that clones of cells in Bloom's syndrome have become homozygous for a particular gene. Somatic cell recombination is proposed as a mechanism to explain the increased incidence of neoplasia in Bloom's syndrome and supports the hypothesis that cancer may be a recessive disorder at the cellular level.

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A 10-year-old boy, who had been in an uninterrupted remission of acute lymphocytic leukemia (ALL) for six years, developed polycythemia vera (PV). One and a half months after detection of PV, he was found to have active leukemia. Both the polycythemia and leukemia receded with anti-leukemia therapy. Three possible explanations for the development of PV in a child with ALL are discussed: 1) PV was a part of his original ALL and recurred whtn patient relapsed. The PV phase was detected only during relapse because the patient was under close observation. 2) PV was a second neoplasm independent of ALL. 3) PV was part of a second leukemia which was different from the original leukemia; this new ALL was derived from a pluripotential cell line involving both erythroid and lymphoid elements. A precedent for this explanation has been observed in chronic myelogenous leukemia.

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Two automatic apparatuses utilizing a dual wave-length spectrophotometric method were developed to perform oxygen dissociation curves on microsamples of blood. This method provides a complete printout of an oxygen dissociation curve in 15 to 20 minutes and the P50 values obtained agree closely with those obtained by classical methods. This technique was used as part of a routine clinical evaluation in assessing oxygen delivery in anemic patients. These apparatuses allowed identification of patients with well-compensated anemia, patients with no compensatory decrease in oxygen affinity, and several patients with a paradoxical increase in oxygen affinity. Knowledge of the oxygen-releasing capacity of the blood permits a more thorough evaluation of oxygen transport in anemic patients than the hemoglobin level and a more precise assessment of the need for transfusion therapy. The ready availability of an oxygen dissociation curve by this method is feasible for routine clinical evaluation and permits more judicious use of blood products.

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Two automatic apparatuses utilizing a dual wavelength spectrophotometer were used to perform oxygen dissociation curves on microsamples of blood. The method provides a complete print-out of an oxygen dissociation curve in 15--20 min and the P50 vlues obtained in normal individuals agree closely with those obtained by classical methods. These apparatuses were used to measure oxygen affinity, ie P50, in anemic children with malignant disease prior to treatment and in children undergoing therapy. Red cell 2,3-DPG levels were also measured. In patients with anemia at the time of diagnosis and prior to therapy, the P50 values and 2,3-DPG levels were elevated as is usual in other types of anemia. However, when oxygen affinity and 2,3-DPG levels were measured in anemic patients receiving treatment, three types of response to anemia were noted: 1) increased P50 and 2,3-DPG; 2) normal or low P50 and 2,3-DPG, and; 3) normal or low P50 with increased 2,3-DPG. Patients who adapted poorly to anemia during treatment had usually received prior intensive chemotherapy and transfusion therapy, and their pattern of red cell glycolytic intermediates was consistent with a red cell population with an increased cell age. The failure of some patients to respond to anemia with a decrease in oxygen affinity has implications in regards to the hemoglobin level at which they should be transfused.

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