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Biomedical subjects

J L Sullivan

Publications and source records attributed to J L Sullivan.

At least 235 records · Page 13Linked to original sources
PubMed1982

Infectious mononucleosis in an adult progressing to fatal immunoblastic lymphoma.

We report a case of infectious mononucleosis progressing to fatal immunoblastic lymphoma. The patient, a 44-year-old man who may have had an immunoregulatory defect, failed to have an appropriate T-cell response to his Epstein-Barr (EB) viral infection. His active EB viral infection was manifest by seroconversion of IgM-viral capsid antibody and a greater than four fold rise in IgG-viral capsid antibody. Also, he transmitted his EB viral infection to his wife who became ill 1 month after his death. Clinically the patient's illness was characterized by waxing and waning lymphadenopathy, persistent fever, diarrhea (similar to that associated with cholera), a coagulopathy, and gastrointestinal bleeding. The patient had pathologic findings of a diffuse immunoblastic lymphoma involving lymph nodes, small bowel, liver, pancreas, kidneys, lungs, and bone marrow. Immunologic cell markers showed the tumor to be polyclonal.

Adult↗
PubMed1981

Iron and the sex difference in heart disease risk.

Premenopausal women in affluent societies are protected from heart diseases which kill large numbers of men. The basis for this sex difference and the loss of protection with menopause is unknown. The hypothesis offered is that the greater incidence of heart diseases in men and postmenopausal women compared with the incidence in premenopausal women is due to higher levels of stored iron in these two groups. The hypothesis is supported by observations of (1) myocardial failure in iron strong disease, (2) accumulation of stored iron with age in men, and (3) accumulation of stored iron after menopause to levels found in men. In addition, the heart diseases of affluence are rare among impoverished peoples who are often iron deficient. The depletion of iron stores by regular phlebotomy could be the experimental system for testing this hypothesis, and a preventive therapy if the hypothesis is confirmed.

Adolescent↗
PubMed1981

Differences in synthesis of membrane proteins by leukemic cells from spleen and peripheral blood indicate distinct subsets of malignant cells in a patient with prolymphocytic leukemia.

Morphological and biochemical differences were demonstrated between prolymphocytic leukemia cells obtained from the spleen and peripheral blood of one patient. Peripheral blood prolymphocytes had consistently smaller nuclear-cytoplasmic ratios than did splenic prolymphocytes. Percoll gradient-purified prolymphocytes from the spleen synthesized abundant amounts of some membrane proteins which were hardly expressed by peripheral blood prolymphocytes. Peripheral blood prolymphocytes did not change their expression of membrane proteins during three days in culture. These findings are consistent with the view that prolymphocytic leukemia cells from the spleen exist, on the average, at an earlier stage of differentiation than do circulating leukemic cells, and that peripheral blood leukemic cells are frozen at a specific phase of differentiation.

Aged↗
PubMed1981

Antibodies to Epstein-Barr virus-specific antigens in patients with hairy-cell leukemia.

Antibody titers to Epstein-Barr virus (EBV)-specific antigens of 19 patients with hairy-cell leukemia (HCL) were markedly elevated. All patients showed high titers of IgG anti-viral capsid antigen (VCA) reactivity equal to or greater than 320 (reciprocal titer). The anti-VCA reciprocal geometric mean titer (GTM) was 1106 in contrast to a GMT of 80 for healthy controls (p less than 0.001). No IgM anti-VCA antibody was detected, but three patients had IgA anti-VCA antibodies. Fourteen patients had elevated anti-early antigen (EA) titers (GMT = 177) which were indicative of active infections with EBV. Seven of the 14 patients demonstrated the diffuse component antibody of the early antigen (EA) complex at a GMT of 98. Anti-Epstein-Barr virus nuclear antigen (EBNA) was detected in sera of 16 patients (GMT = 64, controls GMT = 79). It is noteworthy that three patients with anti-VCA titers lacked anti-EBNA titers. These anti-body responses suggest that immunodeficiency secondary to HCL allowed reactivation of the virus.

Adult↗
PubMed1981

Documentation of Epstein-Barr virus infection in immunodeficient patients with life-threatening lymphoproliferative diseases by clinical, virological, and immunopathological studies.

Multiple methods, pedigree analysis, clinical evaluation, and Epstein-Barr virus (EBV)-specific serology, EBV DNA hybridization of tissues to probe for viral genome, staining of touch imprints for EBV nuclear-associated antigen, establishment of spontaneous infected B-cell lines from peripheral blood or tissues, examination of peripheral blood smears, and hematopathology studies, were used to study seven patients with the X-linked lymphoproliferative syndrome and seven additional patients with life-threatening EBV-associated diseases. These studies demonstrated EBV in the tissues of all 14 patients and immunodeficient antibody responses to EBV were documented. This virus can produce various life-threatening lymphoproliferative diseases in a variety of immunodeficient patients.

Adolescent↗
PubMed1981

Documentation of Epstein-Barr virus infection in immunodeficient patients with life-threatening lymphoproliferative diseases by Epstein-Barr virus complementary RNA/DNA and viral DNA/DNA hybridization.

Tissues from patients thought to have Epstein-Barr virus (EBV)-induced lymphoproliferative diseases were probed for EBV genomes using 2 independent hybridization techniques. Tissues from six patients with the X-linked lymphoproliferative syndrome, all five renal allograft recipients with immunoblastic sarcoma, and eight patients with diverse types of immunodeficiency and lymphoproliferative diseases such as fatal infectious mononucleosis or malignant lymphoma associated with antecedent immunodeficiency contained significant numbers of EBV genome equivalents per cell. The use of 2 hybridization probes is recommended to confirm the presence of EBV genomes. The finding of significant numbers of EBV genomes in tissues from patients with immunodeficiency suggests that EBV is the etiological agent of the associated lymphoproliferative diseases.

Adolescent↗
PubMed1981

Clinical spectrum of lymphoproliferative disorders in renal transplant recipients and evidence for the role of Epstein-Barr virus.

Six renal transplant recipients with abnormal lymphoproliferative disorders were studied in an attempt to define their clinical features and the role of Epstein-Barr virus (EBV) in their pathogenesis. Patients were either teenage (three) or in the sixth decade (three). The younger patients presented an average of 3 months after transplantation with fever, sore throat, and lymphadenopathy; had been markedly immunosuppressed; frequently had preceding or concomitant cytomegalovirus infections; and two of three had a rapidly fatal course. The older patients presented an average of 5 years after transplantation while on maintenance immunosuppressive drugs; in two of three cases with an oropharyngeal tumor; and had a more indolent, but frequently fatal, clinical course. The most frequent sites of biopsy-proven involvement in these patients were lymph nodes (three), the oropharynx (three), liver (three), bone marrow (three), transplanted kidney (three), colon (two), and central nervous system (two). EBV-specific antibody titers including anti-viral capsid antigen IgG, anti-viral capsid antigen IgM, anti-early antigen, and anti-Epstein-Barr nuclear antigen were serially measured in all patients. Four patients demonstrated serological evidence of a primary (one) or reactivation (three) EBV infection. No patient had significant changes in anti-early antigen or anti-Epstein-Barr nuclear antigen titers. All three patients tested for oropharyngeal shedding of EBV were positive. A touch imprint of one tumor was stained for the presence of Epstein-Barr nuclear antigen, and a majority of cells were positive. EBV complementary RNA/DNA filter hybridization and/or viral DNA/DNA reassociation analysis performed on tumor biopsy specimens in five patients demonstrated multiple EBV genome equivalents per cell in all eight specimens tested. Clinical, pathological, serological, and molecular hybridization studies provide substantial evidence that EBV was the cause of these lymphoproliferative disorders occurring after renal transplantation. Impaired host defenses allow the EBV-transformed B-lymphocytes to escape normal control mechanisms. This impairment is invariable and influenced by many factors resulting in the observed spectrum of disease. Cytogenetic changes, however, may also be important.

Adolescent↗
PubMed1981

Primary malignant carcinoid of the testis.

A primary malignant carcinoid in the testis of a 76-year-old man was studied. The clinical indication was the appearance of extensive, papular cutaneous metastases. A markedly enlarged, indurated left testis was discovered during evaluation of the skin lesions. There were no clinical features of carcinoid syndrome. Histochemically, the tumor cells were argyrophilic but nonreactive to an argentaffin stain. Electron microscopy revealed numerous round, neurosecretory-type granules that ranged from 70 to 300 nm. At the time of autopsy, metastases were found in the heart, lungs, and the opposite testis. Review of the laterature failed to uncover any previous reports of a primary malignant carcinoid in the testis.

Aged↗
PubMed1981

The Schick test. A useful tool for screening for antibody deficiency.

The evaluation of suspected humoral immunodeficiency includes quantitative immunoglobulin levels and specific antibody assays. The Schick test is a simple procedure for measurement of specific diphtheria antibodies that has been overlooked because the availability of Schick test materials has been limited in recent years. We assessed the efficacy of the Schick test in screening for antibody deficiency states in 23 children. Five had positive Schick test reactions. All five had clinical and immunologic findings consistent with a primary immunodeficiency disorder. Each of these patients had at least one other abnormality of antibody formation detected. The Schick test is a simple screening device helpful in the assessment of immunocompetence.

Adolescent↗
PubMed1980

Deficient natural killer cell activity in x-linked lymphoproliferative syndrome.

The activity of natural killer cells was found to be deficient in 10 of 12 males with X-linked lymphoproliferative syndrome, a life-threatening proliferation of lymphocytes after infection by Epstein-Barr virus. The activity levels of natural killer cells from affected males were increased after treatment with interferon in vitro, but normal levels of killing were not obtained. Deficient activity of killer cells in individuals with immunodeficiency and chronic infection by Epstein-Barr virus may contribute to the development of lymphoproliferative disorders.

Adolescent↗
PubMed1980

X-linked lymphoproliferative syndrome registry report.

Immune deficiency, especially to the Epstein-Barr virus, and increased susceptibility to fatal infectious mononucleosis, acquired agammoglobulinemia, and lymphoma are the cardinal features of the X-linked lymphoproliferative syndrome. Since the establishment of the XLP Registry in September, 1978, 59 affected males in seven unrelated kindreds were comprehensively studied. A spectrum of lymphoproliferative phenotypes was observed. Thirty-four patients (57%) died from infectious mononucleosis, eight (14%) had fatal infectious mononucleosis with lymphoma (immunoblastic sarcoma), nine (15%) had depressed immunity following EBV infection, and eight (14%) developed lymphoma. Several patients with XLP lacked EBV antibodies despite infection by EBV. The results of this study suggest that EBV can be an oncogenic agent in patients who are immune deficient with XLP.

Adolescent↗
PubMed1980

Metabolic factors affecting monoamine oxidase activity.

While genetics may be the major determinant of monoamine oxidase (MAO) activity, many hormonal, dietary, and even immunological factors may also be important. Knowledge of these factors may be helpful not only in understanding individual variation in MAO levels, but in the application of platelet MAO activity as a clinical laboratory aid.

Adolescent↗
PubMed1980

Electroconvulsive therapy in the presence of brain tumor. Case reports and an evaluation of risk.

The clinical basis for the long established contraindication of electroconvulsive therapy (ECT) in the presence of brain tumor is reviewed, as is the recent literature that has questioned the absolute nature of that contraindication. A need for a specific estimate of risk is noted. Seven retrospective case reports are added to the 28 cases reported in the literature. The clinical case report data are then pooled and evaluated by outcome. Results indicate a 74 per cent overall morbidity, including a 28 per cent 1-month mortality rate for patients with brain tumors who receive ECT. Twenty-one per cent of the patients had a positive behavioral response to ECT without complication.

Adult↗
PubMed1980

Formycin B, purine nucleoside phosphorylase and lymphocyte function.

A genetic deficiency of purine nucleoside phosphorylase (NP) is associated with immune dysfunctin that specifically affects T cells. An absence of adenosine deaminase (ADA), the preceding enzyme in the pathway of purine recycling, is associated with failure of both T and B lymphocyte function. Formycin B, an analogue of inosine and an inhibitor of purine nucleosidase phosphorylase, was used in cell culture systems to stimulate deficiency of this enzyme. The cells used were normal circulating lymphocytes and lymphoblastoid cell lines (LCL) with T and B cell characteristics. Formycin B inhibited the growth of these cells, but its primary effect was found to be due to a mechanism other than purine nucleoside phosphorylase inhibition. The possibility that formycin B was inhibiting adenosine deaminase, for which it is a product analogue, was studied by the analysis of reaction progress curves using the integrated rate equation. The molecular structure of formycin B, whilst preventing its phosphorylytic cleavage by purine nucleoside phosphorylase, could enable this compound to function as a substrate for adenosine kinase. The resultant formation of formycin B nucleotide probably causes the observed inhibition of growth in cultured cells.

Adenosine Deaminase↗