Isolated bone relapse in long-term survivors of acute lymphoblastic leukemia.
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Adolescent↗
Biomedical subjects
B C Lampkin
Publications and source records attributed to B C Lampkin.
Current status of the biology and treatment of acute non-lymphocytic leukemia in children (report from the ANLL strategy group of the Children's Cancer Study Group.
Major advances have been made in the past 10 yr in both the understanding of the biologic characteristics of acute nonlymphocytic leukemia and in the treatment of patients with this disease. Advances in the biologic characteristics include: a better understanding of the nature of leukemic cell proliferation and differentiation; a clearer description of the morphological, histochemical, and ultrastructural characteristics of leukemic cells; a recognition that a high percentage of patients may have specific cytogenetic abnormalities; and a recognition that biochemical differences exist between acute nonlymphocytic leukemia (ANLL) and acute lymphoblastic leukemia (ALL). Today, over 70% of children with ANLL can be induced into a complete remission and over 25% are remaining in a continuous remission for over 2 yr. In spite of these improved results, the best method of extending remissions is unknown. It is unlikely that better results of therapy will be achieved in the future by tailoring the treatment according to the biologic characteristics of the patient, since it appears that ANLL is a heterogeneous group of diseases.
Acute Disease↗
Pediatric hematologic and oncologic emergencies.
Acute hematologic-oncologic problems fall into two groups, those that require immediate assessment, diagnosis, and therapy, and those that require attention but are not life threatening if treated appropriately. Both types are considered in this article, which discusses hemorrhagic disorders; anemias, with special emphasis on patients with sickle cell disease; an approach to fever and infection in the immunocompromised child; and oncologic disorders that may be life threatening.
Anemia, Hemolytic↗
Anthracycline toxicity.
Anthracycline compounds are useful agents in cancer therapy. Major toxicities include mucositis and other gastrointestinal complications, myelodepression and cardiotoxicity. With the current multimodal cancer therapy, anthracycline toxicities may be potentiated by radiation treatment or other chemotherapeutic agents. The choice of anthracycline, drug combination, and drug schedule should be carefully evaluated to minimize toxicity.
Antibiotics, Antineoplastic↗
Transmission of in-vitro radioresistance in a cancer-prone family.
Neoplasms of possible radiogenic origin developed in two members of a family prone to a diversity of cancers, including bone and soft-tissue sarcoma, brain and breast cancers, and leukaemia. Gamma-irradiation survival studies in these two patients and three other relatives, but not their spouses, over three generations demonstrated resistance to cell killing. The D10 value (radiation dose required to reduce survival to 10%) was significantly higher for the five radioresistant strains (491 +/- 30 rad) than for control cultures (405 +/- 18 rad). There was a significant correlation between individual D10 values and D0 survival-curve parameters, indicating that changes in the exponential slope of the survival curves accounted for much of the increase in D10 values. This novel radiation phenotype could be a manifestation of a basic cellular defect, predisposing to a variety of tumours in family members. Thus in-vitro radioresistance, like radiosensitivity, may be a phenotype of a mechanism that increases cancer risk in man.
Adolescent↗
Restoration by normal human immunoglobulin G of deficient serum opsonization for Streptococcus pneumoniae in sickle cell disease.
Supplementation of sera from four children with sickle cell disease with untreated or heated normal human serum or with normal human immunoglobulin G restored opsonic activity for serotype 10 Streptococcus pneumoniae, whereas supplementation with normal human immunoglobulin M did not.
Anemia, Sickle Cell↗
Biochemical and functional abnormalities in lymphocytes from an adenosine deaminase-deficient patient during enzyme replacement therapy.
Biochemical and immunological properties of lymphocytes were measured repetitively over a period of 40 mo during enzyme replacement by transfusion in a child with adenosine deaminase (ADA) deficiency and severe combined immunodeficiency disease. Catalytically defective ADA protein is present in the child's cells. ADA activity in his lymphocytes is 7 nmol/min per 10(8) cells with 51 ng of ADA protein/10(8) cells by radioimmunoassay. ADA activities in normal cord and adult lymphocytes average 193 and 92 nmol/min per 10(8) cells, respectively, with 429 and 223 ng of ADA protein/10(8) cells. Deoxy(d)ATP accumulates in the patient's erythrocytes and lymphocytes. Transfusion of irradiated packed erythrocytes partially corrects the metabolic defects. Frank metabolic relapse occurs if transfusions are discontinued for several months. The amounts of dATP in erythrocytes and lymphocytes averaged 13 and 2 times normal, respectively, during periods when transfusions were administered every 2-4 wk. Deoxyguanosine triphosphate and deoxycytidine triphosphate in lymphocytes were normal on 11 occasions, but deoxyribosylthymine triphosphate was ninefold increased. On 11 occasions dATP was measured in lymphocytes and erythrocytes isolated simultaneously. There was a positive, but statistically insignificant, correlation between amounts of dATP in the two types of cells (r = 0.25,P > 0.1). The absolute peripheral lymphocyte count was correlated with the activity of ADA in circulating erythrocytes and with the response of lymphocytes to phytohemagglutinin (r = 0.64, P < 0.01; r = 0.49, P < 0.05). Response of lymphocytes to stimulation by phytohemagglutinin in vitro and absolute peripheral lymphocyte counts were not significantly correlated with levels of dATP in the erythrocyte or lymphocyte during periods of intensive therapy. Although there was objective improvement during enzyme replacement, the child remained immunodeficient and biochemically abnormal.
Adenosine Deaminase↗
Humoral components of host defense in sickle cell disease during painful crisis and asymptomatic periods.
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Adolescent↗
Clinical and occult testicular leukemia in long-term survivors of acute lymphoblastic leukemia.
Twenty-nine of 60 boys with acute lymphoblastic leukemia survived for more than 30 months and were potential candidates for discontinuation of therapy. Six patients developed overt clinical testicular leukemia: one at 34 months from diagnosis while receiving therapy and five at three to 17 months after therapy was stopped. Elective wedge biopsy of the testes has become part of the evaluation prior to discontinuation of therapy since 1977. Six of 18 boys had microscopic evidence of leukemic infiltration of the testes: four with diffuse involvement and two with focal clusters of leukemic cells. Testicular biopsy is recommended at the time of discontinuation of therapy and perhaps early in the course of the disease, although a negative biopsy does not exclude some focal lesions. The eventual outcome of those with occult testicular leukemia remains to be determined.
Child↗
Effect of 5-fluoro-2'-deoxyuridine on deoxyribonucleotide pools in vivo.
5-Fluoro-2'-deoxyuridine (FdUrd) lowered the dTTP levels in rapidly frozen 12-day W rat embryos and in a human neuroblastoma grown in nude N:NIH(S) mice to about 20% of control values. This effect was associated with greatly increased dCTP levels and reduction of dGTP levels essentially to zero. Elimination of the dGTP pool correlated temporally with the cytotoxicity of FdUrd. Extremely rapid fixation of tissue was required to avoid artifactually high deoxyribonucleoside triphosphate values.
Animals↗
Red-cell-membrane polypeptide aggregates in glucose-6-phosphate dehydrogenase mutants with chronic hemolytic disease. A clue to the mechanism of hemolysis.
Red-cell membranes from patients with glucose-6-phosphate dehydrogenase deficiency were studied with polyacrylamide gel electrophoresis and gel filtration chromatography in sodium dodecyl sulfate. Membranes from each of five such patients who also had chronic hemolytic disease contained polypeptide aggregates within two molecular-weight ranges (4.4 X 10(5) and greater than 50 X 10(6) daltons). The 4.4 X 10(5) dalton aggregates were not detectable in red-cell membranes of patients with the enzyme deficiency without chronic hemolysis or in membranes from normal subjects, and the greater than 50 X 10(6) dalton aggregates were not found in appreciable amounts in these cells. The aggregates were dissociated by mercaptoethanol or dithiothreitol -- indicating that they were formed by intermolecular disulfide bonds. The polypeptide aggregates contained spectrin but not globin. Red-cell deformability was decreased in aggregate-containing cells. We postulate that the polypeptide aggregates are indicators of oxidant damage to the red-cell membrane, which results in decreased deformability and chronic hemolysis.
Adolescent↗
Genealogy of cancer in a family.
Three brothers had separate childhood cancers--osteogenic sarcoma, acute lymphoblastic leukemia, and bilateral malignant neurilemoma. Comprehensive family history showed a total of 16 cases of cancer among the descendants of the proband's great-great-great-grandmother, including a previously unsuspected cluster of similar neoplasms in an distant branch. The constellation of tumors in the family included bony and soft-tissue sarcomas, brain and neural tumors, leukemia, and breast carcinoma, occurring in a pattern suggesting the action of an incompletely penetrant autosomal dominant gene with pleiotropic effects. In some cases the genetic predisposition may have interacted with environmental determinants to produce particular tumors.
Adolescent↗
Immunological and biochemical profiles in response to transfusion therapy in an adenosine deaminase-deficient patient with severe combined immunodeficiency disease.
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Adenosine Deaminase↗
Emergency splenectomy for idiopathic thrombocytopenic purpura in children.
Splenectomies were performed in 30 children out of a total of 183 with idiopathic thrombocytopenic purpura (ITP). Of these 30, ten splenectomies were performed on an emergency basis. Significant spontaneous gross hematuria, gastrointestinal bleeding or epistaxis appear to represent harbingers of intracranial hemorrhage and constitute indications for emergency splenectomy.
Adolescent↗
Acute respiratory illness in children with acute lymphoblastic leukemia.
Ten of 70 children (14%) with acute lymphoblastic leukemia developed severe interstitial pneumonitis within three weeks after induction of central nervous system prophylactic therapy. The clinical picture was characterized by fever, cough, progressive dyspnea, and hypoxemia with complete resolution in one to three weeks, except in one patient who died during the acute illness from respiratory failure. P. carinii organisms were found in the lung tissue of only one patient. The etiology of the pneumonitis in the other nine children was probably viral, acquired or activated during a period of lymphopenia and immunosuppression. The morbidity and potential mortality from the pneumonitis warrants early recognition by open lung biopsy and intensive supportive therapy.
Central Nervous System Diseases↗
Bone marrow cellularity determination: comparison of the biopsy, aspirate, and buffy coat.
Bone marrow biopsies (244) performed with a Jamshidi needle were evaluated in 53 children with leukemia or aplastic anemia. Adequate specimens were obtained in 85%. Results of cellularity estimated by biopsy were compared to the cellularity of the aspirate versus volumetric determination of the myeloid-erythroid layer (buffy coat). A wide discrepancy was noted between marrow cellularity confirmed by biopsy versus the aspirate or buffy coat. The greatest variance was seen in the hypercellular or normocellular marrows, as estimated by biopsy, in which 39% were misinterpreted as moderately or severely hypocellular by aspirate. Volumetric measurement of buffy coat was least acceptable for estimating cellularity. Thus the biopsy has proved to be an important and reliable indicator of bone marrow cellularity.
Anemia, Aplastic↗
Surface markers and prognostic factors in acute lymphoblastic leukemia.
We investigated the surface markers on lymphoblasts from 37 patients with acute lymphoblastic leukemia. Spontaneous rosette formation with sheep erythrocytes (E rosettes) identified T cells and the presence of surface immunoglobulin identified B cells. Eight patients had T-marker lymphoblasts; 28 had no markers (null lymphoblasts), and one patient had B-marker lymphoblasts. The eight patients with T-marker acute lymphoblastic leukemia had massive leukemic infiltration, frequently a mediastinal mass, and a poor prognosis. The T-marker lymphoblasts had a weak or negative periodic acid-Schiff reaction and a very low antigenic stimulation to allogeneic lymphocytes. The association of T-marker lymphoblasts and some of the conventional poor-risk factors appears to be reliable in predicting a poor prognosis.
B-Lymphocytes↗
Lymphocyte subpopulations in pertussis.
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B-Lymphocytes↗