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Biomedical subjects

F B Ruymann

Publications and source records attributed to F B Ruymann.

At least 55 records · Page 3Linked to original sources
PubMed1984

Bone marrow metastases at diagnosis in children and adolescents with rhabdomyosarcoma. A report from the intergroup rhabdomyosarcoma study.

Thirty children and adolescents with rhabdomyosarcoma (RMS) entered on IRS-1 in a 5-year period had bone marrow metastases (BMM) at diagnosis. BMM at diagnosis occurred in 30/103 patients (29%) with Clinical Group IV RMS. On the average, patients with BMM were older with more extremity primaries than other IRS-1 patients. Alveolar histology was present in 15/30 patients (50%) with BMM, compared to 19% on IRS-1. Eleven patients received vincristine, dactinomycin, cyclophosphamide (VAC), while 19 patients received VAC with Adriamycin (ADR). The overall complete response (CR) rate was 60%. The median disease-free survival times by treatment were 17 weeks with VAC and 32 weeks for VAC + ADR (P greater than 0.10). Relapse from CR in Clinical Group IV patients with BMM occurred in 16/18 patients (89%) and was significantly higher than in 17/29 Clinical Group IV patients (59%) without BMM (P less than 0.035). BMM at diagnosis in children and adolescents with RMS is frequently associated with alveolar histology and is a poor prognostic sign.

Adolescent↗
PubMed1984

Effect of retinoic acid on the clonal growth of childhood myeloid and lymphoid leukemias: a pediatric oncology group study.

We have studied the effects of retinoic acid (RA) on bone marrow leukemic cells from children with acute nonlymphocytic leukemia (ANLL) at the time of diagnosis, and on cells from four ALL/lymphoma cell lines (common-ALL, pre-B-ALL, T-ALL, and Burkitt's lymphoma) derived from children with these diseases. Cells were cultured in methylcellulose medium with clinically attainable concentrations (0.25-2.0 microM) of RA for two weeks prior to colony and cluster quantitation. Myeloid progenitor cells (CFU-GM) obtained from children with hematologically normal bone marrows were also cultured with RA. Of 19 patients with ANLL whose cells formed colonies, 16 (84%) were inhibited by RA; three patients showed either increased or unchanged colony numbers with RA. RA had a similar effect on both ANLL cluster and colony growth. RA (1-2 microM) also inhibited colony growth of the pre-B-ALL, common-ALL, and Burkitt's lymphoma lines; the T-ALL line and normal bone marrow CFU-GM were not inhibited. The inhibitory effects of RA on pediatric ANLL bone marrow cells and on some ALL/lymphoma cell lines compared with CFU-GM indicate that RA may be of value in the treatment of these malignancies in children.

B-Lymphocytes↗
PubMed1983

Hepatic failure secondary to indicine N-oxide toxicity. A Pediatric Oncology Group Study.

Indicine N-oxide, a pyrrolizidine alkaloid, was given to a five-year-old boy with refractory acute myelocytic leukemia. Three days after receiving the drug the patient developed signs and symptoms of acute hepatic failure. The patient died nine days after receiving the drug and an autopsy showed massive hepatic necrosis. The acute hepatic failure observed in this patient may have been secondary to indicine N-oxide toxicity.

Antineoplastic Agents, Phytogenic↗
PubMed1983

Newborn polymorphonuclear leukocyte aggregation: a study of physical properties and ultrastructure using chemotactic peptides.

N-formyl-L-methionyl-L-leucyl-L-phenylalanine (FMLP) and N-formyl-L-methionyl-L-phenylalanine (FMP) were used to investigate neutrophil (PMN) aggregation. Neutrophils were isolated from healthy adult volunteers and term newborn cord blood. Neutrophil aggregation was measured after the addition of FMLP and FMP. Adult PMN aggregation curves demonstrated initial aggregation with slow deaggregation. Newborn neutrophil aggregation curves showed slow aggregation with no deaggregation. These results were identical to the adult and newborn neutrophil aggregation curves produced by C5a. Newborn PMN aggregates examined by scanning electromicrography showed frequent, dense aggregates compared with fewer, less dense aggregates of adult PMNs. Adult and newborn PMN aggregates differed when compared by transmission electromicrographs (EM). Newborn PMNs were tightly bound with cell membrane projections; adult PMNs were loosely bound with no cell membrane projections. Cytochalasin-B pretreated adult and newborn PMN aggregates displayed close approximation of cell membranes with large numbers of cytoplasmic projections. Newborn neutrophils are irreversibly aggregated by FMLP and FMP whereas adult neutrophils display an aggregation-deaggregation reaction. EM studies suggest that this irreversible aggregation of untreated newborn neutrophils may differ from the irreversible aggregation of cytochalasin-B pretreated neutrophils.

Adult↗
PubMed1982

Soft-tissue sarcoma of the trunk in childhood. Results of the intergroup rhabdomyosarcoma study.

Disease-free survival data were obtained on 30 children with soft-tissue sarcoma of the trunk. The children were clinically grouped and treated in accord with the IRS protocol (Cancer 1977; 40:2015) from November 1972 through December 1976. Histologically, 40% of the tumors were alveolar rhabdomyosarcoma (RMS), 20% were embryonal RMS, 20% were extraosseous Ewing's sarcoma, 17% were undifferentiated sarcoma, and 3% were pleomorphic RMS. In 13 patients with primary tumors of the chest wall, the male-to-female (M:F) ratio was 3:11, and the median age was 12.5 yr. Five of the ten patients (50%) with localized tumor (Groups I-III) were disease-free at a median of 4.7 yrs. after diagnosis; two died of locally recurrent tumor, two died of metastases to lungs or bones, and 1 died of intracranial hemorrhage. None of the four with chest wall tumors and distant metastases (Group IV) survived. In ten patients with localized paraspinal primary tumors, the M:F ratio was 8:2, and the median age was 3.5 yrs. None had metastases. Seven of ten (70%) were disease-free at a median of 4.7 yrs. after diagnosis. Two died after regional recurrence developed (one retroperitoneal, one meningeal), and one died of lung metastases. In six patients with primary tumors of the abdominal wall, the M:F ratio was 3:3 and the median age was 9.5 yrs. Three of five with localized tumor (Groups I-III) were disease-free at a median of 5 yr. after diagnosis; one died after regional recurrence, and one died from accidental trauma. The one Group IV patient died of tumor. Overall, 15 of 30 patients (50%) are alive and free of recurrent disease at a median of 5+ yr. after initiation of treatment. The authors conclude that prognosis is most favorable for patients with paraspinal tumors, least favorable for those with chest wall tumors, and intermediate in patients with abdominal wall tumors. Prognostic differences appeared to be influenced by extent of disease at diagnosis and histologic subtype of the sarcoma.

Abdominal Neoplasms↗
PubMed1982

Cardiomyopathy in a child with hypereosinophilic syndrome.

A 9-year-old boy presented with increasing fatigue, anorexia, weight loss, fever, and absolute eosinophilia (48,000/microL). Pulmonary infiltrates occurred 3 months later. A murmur of mitral regurgitation was heard 5 months after onset of illness, and heart failure soon followed. Despite corticosteroid therapy the eosinophilia persisted intermittently until 1 month before death. The patient died within 9 months of the onset of illness. At necropsy there was cardiomegaly with subendocardial fibrosis in the right and left ventricles. Thrombi were present in the left ventricular apex and behind the posterior mitral leaflet. The findings in 12 previously reported pediatric cases are reviewed. The etiopathogenesis of the hypereosinophilic syndrome is discussed: half of the cases in children are associated with leukemia.

Cardiomyopathies↗
PubMed1981

Second malignancy in acute lymphocytic leukemia. Review of 33 cases.

Improved survival in childhood cancer resulting from advances in therapy is frequently associated with long-term morbidity, including the potential for second malignancy. In a review of the literature in the English language, we found 33 cases of acute lymphocytic leukemia (ALL) followed by a second neoplasm. Second tumors in this group of patients with ALL include seven cases of histiocytic medullary reticulosis, four cases of Hodgkin's disease, nine cases of acute myelocytic leukemia, four cases of chronic myelocytic leukemia, and nine cases of solid tumors. The appearance of subsequent malignancies may be related to a combination of therapy-induced immunosuppression, a direct carcinogenic effect resulting from therapy, and/or constitutional factors. Physicians should be alert to the possibility of subsequent malignant neoplasms in survivors of cancer.

Adolescent↗
PubMed1980

Decreased phytohemagglutinin-induced aggregation and C5a-induced chemotaxis of human newborn neutrophils.

Phytohemmagglutinin (PHA)-induced lectin aggregation, chemotactic response to C5a, and random migration were measured on paired samples of neutrophils obtained from human peripheral blood and cord blood of normal newborn infants. The mean aggregation rate (+/- 1 SD) of adult neutrophils with PHA was 16.8 +/- 4.4 vs. 12.0 +/- 3.6 for newborn neutrophils (P less than 0.005), and the mean percent aggregation of adult neutrophils was 56.2 +/- 9.2 vs. 45.6 +/- 8.3 for newborn neutrophils (P less than 0.005). Exposure to newborn plasma had no affect on adult neutrophil aggregation. Whereas vinblastine (VBL) decreased both the percent and rate of PHA-induced adult neutrophil aggregation, only the rate of newborn neutrophil aggregation was reduced by VBL. Newborn neutrophil chemotactic response to C5a, was reduced by 80% (P less than 0.0025), and showed a positive correlation with percent PHA-induced aggregation (r = 0.6037, P less than 0.05). On the other hand, random migration was not significantly reduced and did not correlate with PHA-induced aggregation. These observations suggest that the decreased chemotactic responsiveness of newborn neutrophils may be due to developmental membrane differences which adversely affect the number and/or availability of C5a resceptors. Lectin-induced aggregation studies of other chemotactic defects may identify similar differences.

Cell Aggregation↗
PubMed1979

Anthracycline cardiomyopathy in children: report of two cases.

Two children with leukemia are presented, each demonstrating an unusual aspect of anthracycline-induced cardiomyopathy. In the first patient (a 7-month-old female with acute monocytic leukemia) extremely young age and previous chemotherapy with a podophyllotoxin derivative, VP-16, may have prediposed the patient to fatal congestive heart failure at a total Daunorubicin dose of only 225 mg/m2. In the second patient, a delay of 4 1/2 years was not sufficient in preventing congestive heart failure resulting from the administration of additional anthracycline chemotherapy. We conclude that extremely young age and, possibly, prior therapy with VP-16 may be addition risk factors in the development of anthracycline cardiomyopathy. Also, we suggest that once an anthracycline agent has reached a toxic threshold for the myocardium, the heart may always be at risk to injury from additional Adriamycin or Daunorubicin therapy.

Child↗
PubMed1979

Evaluation of intensified chemotherapy in children with advanced rhabdomyosarcoma (clinical groups III and IV). The Intergroup Rhabdomyosarcoma Study (IRS) Committee of the Cancer and Leukemia Group B Children's Cancer Study Group, Southwest Oncology Group.

Twenty-seven previously untreated children with gross residual (20) or metastatic (seven) rhabdomyosarcoma were treated with pulse-VAC (vincristine weekly for 12 doses plus dactinomycin and cyclophosphamide simultaneously given daily for 5 days) and radiotherapy. Toxicity during the 12-week induction period included 23 of 27 (85%) with an absolute neutrophil count (ANC) under 500/mm3; 16/27 (59%) were given intravenous (I.V.) antibodies. Three patients developed Gram-negative sepsis and two of them died. In the first 12 weeks, eight children had a complete response (CR) and another 10 a good partial response (PR), a total of 18 of 27 favorable responses (67%). At 12 weeks, 20 patients received either intermittent pulse-VAC (Regimen H) or a pulse of adriamycin plus vincristine and cyclophosphamide alternating with pulse-VAC (Regimen I) every 4--6 weeks. After this first "maintenance," only seven patients (35%) developed an ANC under 500/mm3 and only three (15%) were given I.V. antibiotics. Severe toxicity disappeared with drug dose reduction in subsequent courses. The overall CR rate was 59% with a PR rate of 15%, a total of 74% favorable responses. This rate is not significantly better than that obtained by previous IRS chemotherapy and radiotherapy schedules for patients with gross residual and metastatic rhabdomyosarcoma. Future studies in these patients will concentrate on diminishing myelosuppression while shortening the rest period between pulses, in order to deliver more drug per unit time.

Adolescent↗
PubMed1978

Splenic sequestration and ineffective erythropoiesis in hemoglobin E-beta-thalassemia disease.

A 13-year-old Thai female with hemoglobin E-beta-thalassemia was evaluated for anemia and splenomegaly. Globin chain synthesis in a whole cell system revealed an absence of betaA chains and excessive alpha chains. The alpha/betaE + gamma ratio was 1.26 in bone marrow and 1.90 in peripheral blood. The average gamma/betaE ratio in bone marrow and peripheral blood was 0.36 compared to peripheral blood concentrations of 49% hemoglobin E and 51% hemoglobin F. Homologous red cell 51Cr half-life increased from 22.7 days to 32.8 days after splenectomy. Total circulating hemoglobin increased from 112.9 to 149.7 g. Endogenous carbon monoxide productive (Vco) as a measure of total heme catabolism decreased from 2.00 to 1.54 mumol/hr/kg. Ineffective erythropoiesis was manifested by an increased Vco/Vheme-c ratio of 7.52.

Adolescent↗
PubMed1977

Hepatoma in a child with methotrexate-induced hepatic fibrosis.

During autopsy, an 11-year-old girl with acute lymphocytic leukemia was found to have hepatic fibrosis with a 2.5-cm nodule of hepatocarcinoma. For 5 1/2 of the six years of treatment, chemotherapy consisted of daily orally administered methotrexate with monthly doses of vincristine sulfate and prednisone. The cumulative dose of methotrexate was 2.5 g. To our knowledge, this report constitutes the first association of hepatoma with methotrexate-induced hepatic fibrosis. A careful follow-up of patients receiving methotrexate for malignant neoplasms is urged; caution is suggested in the prolonged use of methotrexate when treating benign disease.

Carcinoma, Hepatocellular↗
PubMed1977

Granulocyte transfusion therapy in children.

A prospective study of 45 granulocyte transfusions in children using continuous flow centrifugation is reported. During 13 episodes of proven or presumed infection, only two children failed to show a favorable response to granulocyte transfusion. The neutropenic child shows a significantly increased absolute granulocyte count one hour after transfusion. The granulocyte counts at one hour after transfusion are inversely proportional to the child's size. A child with chronic granulomatous disease who had documented Nocardia asteroides sepsis and pneumonia exhibited complete recovery following granulocyte transfusion. Dramatic responses to the nonrandom use of granulocyte transfusion have been observed in children with major gram-negative bacterial infections. Endorsement of granulocyte transfusion for instances of presumed, but unproven, infection in the neutropenic child will require randomization to control the variables of antibiotic therapy and bone marrow remission.

Bacterial Infections↗