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Biomedical subjects

R R Jones

Publications and source records attributed to R R Jones.

At least 55 records · Page 3Linked to original sources

Analysis of beta, gamma, and delta T-cell receptor genes in mycosis fungoides and Sezary syndrome.

The authors have analyzed the configuration of immunoglobulin (Ig) and beta, gamma and delta T-cell receptor (TCR) genes in DNA extracted from skin, lymph nodes, and peripheral blood mononuclear cells obtained from 41 patients with mycosis fungoides (MF), 14 patients with Sezary syndrome, and 13 patients with benign inflammatory dermatoses. No discrete rearranged bands (DRB) were detected in patients with inflammatory dermatoses. In tissue DNA from 19 patients with MF DRB were detected with beta and gamma, but not delta TCR probes. Only one patient with MF had a rearrangement of gamma and delta with germ line beta TCR genes. In 13 patients multiple biopsies were analyzed and DRB, when present, were identical in different lesions from individual patients. In three patients analysis of DNA from dermatopathic lymph nodes did not reveal DRB. Analysis of peripheral blood DNA from 24 patients revealed a discrete rearrangement of the gamma TCR gene in four patients and both beta and gamma genes in four additional patients. In MF DRB were detected more frequently with advancing stage of disease in tissues (P less than 0.01) but not in peripheral blood (P equals 0.36). Of 14 patients with Sezary syndrome, eight had DRB in peripheral blood DNA with both beta and gamma probes and in three of these patients identical DRB were also detected in DNA from skin biopsy samples. In contrast, DRB were not detected in the peripheral blood of the other six patients. In both MF and Sezary syndrome there was no restricted usage of particular V gamma genes. These results indicate that in MF (1) T-cell clones can be detected in skin biopsy specimens from the majority of patients with early stage disease, (2) gamma delta T-cell clones are only rarely found, and (3) TCR gene analysis can detect T-cell clones in the peripheral blood with a greater degree of specificity than conventional light microscopic study. In Sezary syndrome these studies also suggest that a subset of patients have a polyclonal population of circulating atypical lymphoid cells. In addition these patients appear to have a better prognosis than those with monoclonal disease.

DNA, Neoplasm↗

Analysis of beta, gamma, and delta T-cell receptor genes in lymphomatoid papulosis: cellular basis of two distinct histologic subsets.

To investigate the histogenesis of lymphomatoid papulosis (LYP), we have analyzed the configuration of the beta, gamma, and delta T-cell receptor (TCR) and Ig genes in DNA from 31 biopsies of 18 patients with this condition and also from peripheral blood mononuclear cells of eight of these patients. Immunoglobulin genes were in a germ-line configuration in all patients, and TCR genes were in a germ-line configuration in six patients. In nine patients, one or two rearranged bands (RB) were detected with both beta and gamma TCR probes and in one patient with beta, gamma, and delta TCR probes. Two patients, in whom beta and delta TCR genes were in a germline configuration, had evidence of multiple discrete rearrangements of gamma TCR genes, consistent with a polyclonal T-cell population. Analysis of multiple biopsies revealed that RB, when present, were identical in different lesions from individual patients. All but one of the peripheral blood samples showed a germ-line configuration. The exception had evidence of a rearrangement of gamma and delta in peripheral blood and a beta and gamma rearrangement within tissue. This study has established that only a proportion of patients with LYP have a monoclonal T-cell proliferation. Correlation with the clinicopathologic and immunophenotypic data revealed that a T-cell clone was limited to patients with Willemze type B LYP or "mixed type" LYP, whereas patients with type A LYP consistently showed a germline configuration of TCR genes. This study indicates that in LYP the atypical hyperchromatic cerebriform mononuclear cells of type B invariably constitute a monoclonal T-cell population whereas the atypical CD30 positive type A cells represent a proliferation of cells of non-B, non-T-cell lineage.

Adult↗

Lupus erythematosus profundus with partial C4 deficiency responding to thalidomide.

A female patient with disfiguring lupus erythematosus profundus (LEP) from the age of 13 years was found to have an isolated partial C4 deficiency, with reduced levels of both allotypes, C4A and C4B. A genetic basis for the hypocomplementaemia was confirmed by a family study of complement and HLA types which revealed heterozygous null alleles for C4A and C4B in the proband. Marked improvement in her cutaneous lesions occurred with thalidomide.

Adolescent↗

Yellow nail syndrome in association with carcinoma of the gall bladder.

We report the case of a 54-year-old female who was found to have adenocarcinoma of the gall bladder 2 years after the onset of yellow discolouration and deformity of all 20 nails. She also exhibited a past history of chronic sinusitis and mild bronchiectasis. As the tumour was inoperable, palliative surgery was performed. This case adds further support to a possible association between malignancy and the yellow nail syndrome (YNS).

Adenocarcinoma↗

Plasma exchange in refractory cutaneous vasculitis.

Eight patients with intractable cutaneous leucocytoclastic vasculitis were given a trial of plasma-exchange therapy. All but one improved, five substantially. Four have been successfully treated by intermittent plasma exchange for periods of 5-12 years. Apart from one episode of hepatitis B, possibly related to administration of fresh frozen plasma, no major adverse effects have occurred. Plasma exchange can be a valuable therapeutic option in patients with severe refractory cutaneous vasculitis.

Adult↗

Rash after treatment with anistreplase.

A palpable purpura developed on the legs and lower abdomen of a woman of 54 five days after she was treated with anistreplase anisoylated plasminogen streptokinase activator complex (APSAC) for an acute myocardial infarction. Histological examination of a skin biopsy specimen taken 6 days after treatment showed leucocytoclastic vasculitis. The rash resolved within two weeks and there were no other complications.

Anistreplase↗

The history, chemistry and pharmacokinetics of Sanguinaria extract.

Sanguinaria extract is a mixture of benzophenanthridine alkaloids derived from Sanguinaria canadensis L. (bloodroot). This mixture of alkaloids has a long history of use in tinctures and expectorants in pharmaceutical products. The purity of Sanguinaria extract is well defined. The chemistry and biochemistry of these alkaloids, including the dynamic equilibrium between acid and base forms, and pharmacokinetics of Sanguinaria extract shall be presented when this extract is incorporated into a dentifrice or oral rinse formulation.

Alkaloids↗

Radiation limits.

Explore the source record for details and available documents.

Environmental Exposure↗

Solitary morphoea profunda.

This study describes a series of patients with an unusual form of scleroderma. All five patients presented clinically with solitary, indurated plaques involving the upper trunk. Histologically the lesions were characterized by a dense, mononuclear cell infiltrate mainly in the subcutis, which was rich in plasma cells, and was associated with marked sclerosis and hyalinization of the connective tissue at this level. Lymphoid aggregates with germinal centre formation were present in three patients and tissue eosinophilia in one. No lesion has shown spontaneous resolution but neither has systemic involvement occurred, despite a maximum follow-up of 17 years. Immunohistochemical studies revealed a mixed population of T and B lymphocytes with no evidence of immunoglobulin light-chain restriction. We believe that the clinical and histological features in these five cases are sufficiently distinct to merit description under the title of 'solitary morphoea profunda'.

Adult↗

The histogenesis of mammary and extramammary Paget's disease.

The histogenesis of mammary and extramammary Paget's disease has been studied by immunohistochemical staining of paraffin-embedded tissue using a panel of epithelial cell markers, which react with secretory or ductal epithelium, but not stratified epithelium. These markers included a monoclonal antibody E29 to epithelial membrane antigen EMA, the cytokeratin marker CAM 5.2 and three new monoclonal antibodies raised to human milk fat globule membrane (LICR-LON-TW19 and H.10.A) and a human bladder cell cancer line (3.77). The findings demonstrate that both mammary and extramammary Paget's disease are of epithelial cell origin and share antigens expressed by simple epithelia. Some antigens, such as EMA and low molecular weight cytokeratins are consistently present in both diseases, whereas other antigens, identified by H.10.A and TW19 are found more frequently in cases of extramammary Paget's disease. This panel of monoclonal antibodies also proved useful in distinguishing Paget's disease from pagetoid melanoma and clear cell Bowen's disease.

Adult↗

Depth and area of dental erosions, and dental caries, in bulimic women.

Dental caries and the severity (depth) and area of erosions of tooth substance were studied in 11 bulimic subjects and 22 matched controls. Erosions were seen in 69% of the bulimics' teeth and in only 7% of the controls' (p less than 0.001). Depth of penetration was greater in the bulimics, extending even into the pulp, and the area of erosion was also larger. Scores for dental caries were higher in bulimics (mean DMFS 27.9, median 36) than in controls (mean DMFS 19.1, median 13.5), although the difference was not statistically significant. This study suggests that when erosions are present on the lingual surfaces of maxillary anterior teeth, as well as on the buccal surfaces of maxillary canines, premolars, and maxillary incisors, a diagnosis of bulimia is likely. Patients with these signs should be referred for medical help.

Adult↗

Lymphomatoid papulosis and its relationship to "idiopathic" hypereosinophilic syndrome.

Persistent hypereosinophilia, endomyocardial fibrosis, and a recurrent self-healing papulonodular eruption with the histologic features of lymphomatoid papulosis are described in three patients. One patient died after developing an acute myeloblastic transformation in the eosinophil series. Immunocytochemical studies of cutaneous lesions in two of the patients suggested a mature T-cell phenotype with a predominant population of CD4-positive cells. Immunostaining of cutaneous tissue with monoclonal antibodies BE1 and BE2 yielded negative findings. Because it is now known from in vitro studies that T lymphocytes secrete the eosinopoietic factor, interleukin 5, it is possible that the cutaneous lesions, hypereosinophilia, and associated endomyocardial fibrosis were induced by transformed helper T lymphocytes in these three patients.

Eosinophilia↗

Teaching pathology in the 21st century. An experimental automated curriculum delivery system for basic pathology.

In late 1984, the "General Professional Education of the Physician" (GPEP) report recommended, among other things, that medical curricula be revised to rely less on lectures and more on independent study and problem solving. We seem to have anticipated, in 1980, the findings of the GPEP panel by formulating and starting to test the hypothesis that certain "core" information in medical curricula can be as effectively delivered by technology-based self-study means as by lecture or formal laboratory. We began, at that time, to prepare a series of self-study materials using, at first, videotape and then computer-controlled optical videodiscs. The content area selected for study was basic microscopic pathology. The series was planned to cover the following areas of study: cellular alterations and adaptations, cell injury, acute inflammation, chronic inflammation and wound healing, cellular accumulations, circulatory disturbances, necrosis, and neoplasia. All are intended to provide learning experiences in basic pathology. The first two programs were released for testing in 1983 as a two-sided videodisc accompanied by computer-driven pretests, study modules, and posttests that used Apple computers and Pioneer (DiscoVision) videodisc players. An MS DOS (eg, IBM) version of the computer programs was released in 1984. The first two programs are now used in 57 US, Canadian, European, and Philippine health professions schools, and over 1300 student and faculty evaluations have been received. Student and faculty evaluations of these first two programs were very positive, and, as a result, the others are in production and will be completed in 1988. Only when a critical mass of curriculum is available can we really test our stated hypothesis. In the meantime, it is worthwhile to report the evaluation of the first two programs.

Audiovisual Aids↗