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S Whittaker

Publications and source records attributed to S Whittaker.

At least 37 records · Page 2Linked to original sources

Sézary syndrome: diagnostic criteria and therapeutic options.

Sézary syndrome (SS) is a rare form of erythrodermic cutaneous T-cell lymphoma with hematological involvement and a poor prognosis. Therapies include photopheresis, with or without interferon, chemotherapy, and total skin electron beam therapy. The lack of any randomized studies makes it difficult to assess the effect of current therapy on survival. In addition, the different response rates reported for individual treatments may depend as much on the criteria used to define SS as the therapy itself. This article reviews the diagnostic tests that are needed to reliably diagnose SS and offers a critical analysis of current treatment options.

Antineoplastic Agents↗

Adjuvant therapy in melanoma.

Melanoma is an immunologic tumour as indicated by clinical regression, long dormancy and the presence of class 1 dependent cytotoxic responses against well defined tumour peptides. The poor prognosis and relative chemoresistance of patients with regional nodal or metastatic disease highlights the urgent need for an effective adjuvant therapy. A wide variety of different agents have been assessed including high dose interferon which has been shown to improve overall survival, although results of a subsequent study have not confirmed these findings. Currently, a variety of different biotherapies and biochemotherapy regimes are being assessed in phase II and III studies and sentinel lymph node biopsy now provides an accurate method for staging so that all patients can be stratified into well-designed randomised controlled trials.

Adjuvants, Immunologic↗

Scleromyxedema: response to high-dose intravenous immunoglobulin (hdIVIg).

We report 2 patients with scleromyxedema, both associated with IgG-lambda paraproteinemia, who were treated with high-dose intravenous immunoglobulin (hdIVIg) 2g/kg per month. The response to treatment was assessed using an objective skin scoring system initially established for patients with scleroderma. This system grades the overall severity of the induration and the reduction in mobility of the skin. Both patients initially had a dramatic response to treatment which was sustained in one patient. The first patient, a 30-year-old black man, showed a reduction in skin scores from 36/60 to 11/60 over a 3-month period, during which time he had 3 infusions of hdIVIg. After an unplanned 2-month break from treatment, severe neuromuscular complications developed. These improved initially with more frequent infusions of hdIVIg but oral corticosteroids were required to treat worsening myopathy. Unfortunately, the initial response to hdIVIg has not been sustained and his skin scores at 1 year returned to baseline. The second patient, a 60-year-old white man, showed a similarly dramatic reduction in skin scores from 36/60 to 15/60 over a 3-month period after having received only 2 infusions of hdIVIg. There has been sustained improvement after 10 months of therapy and the interval between hdIVIg infusions has been increased to 10 weeks without deterioration. HdIVIg may be an effective treatment for some patients with scleromyxedema, a rare condition with few effective treatments and a poor prognosis.

Adult↗

Status of a health care quality review programme in South Africa.

This paper provides an overview of an accreditation programme for health care facilities in South Africa. It traces the origin of COHSASA (The Council for Health Service Accreditation of Southern Africa) which began as a pilot programme in 1992, to its current status as the only accreditation body in the country. There are brief descriptions of its structure, how it is governed, and how standards were developed and organized. The authors sketch a background of the unique sociopolitical context and legal developments within which the programme operates in South Africa and how the programme is contributing towards the new government's intention to provide equitable and quality health care to all its citizens. There is an outline of the principles on which the COHSASA programme is based and the structure and process of the programme. The programme incorporates an integrated, multi-disciplinary, continuous quality improvement approach with special emphasis on capacity building of hospital staff when necessary. The paper refers to groundbreaking research in Kwa-Zulu Natal where the impact of accreditation is being measured in a randomized control trial. It points to the benefits of accreditation being perceived in both public and private sectors of health care in South Africa and outlines some of the results of the program's implementation.

Accreditation↗

T-cell receptor gene analysis in the diagnosis of Sézary syndrome.

Diagnosing Sézary syndrome (SS) on clinicopathological grounds alone is far from straightforward, particularly in the early stages of the disease. Atypical lymphocytes may be seen in the peripheral blood of patients with reactive forms of erythroderma, so additional criteria are needed to establish the diagnosis of a T-cell leukemia/lymphoma. A wide variety of confirmatory tests have been proposed in the literature, but there has been no systematic attempt to compare the specificity and sensitivity of these different methods. Recent data indicate that T-cell receptor (TCR) gene analysis is the most useful test currently available and that methods based on polymerase chain reaction are more sensitive than Southern blot analysis. We propose that the diagnostic criteria for SS should include erythroderma, atypical circulating mononuclear cells, and evidence of a clonal T-cell population in the peripheral blood. Clonality can be established with certainty by cytogenetic or TCR gene analysis, but only the latter is sufficiently sensitive to be of value in routine diagnosis. Immunophenotypic data showing an expanded CD4(+)/CD7(-) population, an elevated CD4/CD8 ratio, or restricted V beta expression are not specific to T-cell malignancy and should not be used as a sole diagnostic criteria in SS. Entry criteria for future clinical studies will need to be more rigorous if meaningful comparisons are to be made between different treatment options.

Genes, T-Cell Receptor beta↗

Necrobiotic cutaneous T-cell lymphoma.

We report 3 patients with granulomatous cutaneous T-cell lymphoma (CTCL) who showed necrobiosis histologically with palisading granulomas. Although granulomatous change may be present in up to 4% of cases of CTCL, necrobiosis is rare. Misdiagnosis may occur if epidermotropism is minimal or if atypical cells are masked by the granulomatous infiltrate. T-cell receptor gene analysis confirmed the presence of clonal T-cell populations in lesional skin from all 3 cases.

Adult↗

A non-comparative multi-centre clinical evaluation of a new hydropolymer adhesive dressing.

This study evaluates the performance of a new hydropolymer adhesive dressing in the treatment of chronic or acute wounds, in the presence of blood and/or low levels of exudate, in 74 patients, in a five-site multi-centre non-comparative clinical trial of four weeks' duration. The primary efficacy variable was defined as the incidence of central island dressing adherence to the wound bed in the final stages of healing. The hydropolymer dressing performed extremely well with 98.4% (240 out of 244) of the dressings not adhering to the wound bed.

Acute Disease↗

Dermatological uses of high-dose intravenous immunoglobulin.

High-dose intravenous immunoglobulin (hdIVIg) is increasingly used to treat a range of inflammatory and autoimmune diseases. The current dermatological uses of hdIVIg include the treatment of dermatomyositis and the autoimmune bullous disorders, epidermolysis bullosa acquisita, pemphigoid, and pemphigus. Numerous immunomodulatory mechanisms for hdIVIg have been proposed, and they are discussed alongside treatment protocols and adverse effects. Increasing use of this therapy has helped to establish its excellent safety record, without the many adverse effects of steroids and other immunosuppressive agents. This safety record makes hdIVIg an attractive therapeutic option; however, in view of the time required to administer the infusions, the cost, and the urgent need for controlled trials of hdIVIg in patients with specific dermatological disorders such as pemphigus, patients must be carefully selected. Unfortunately, current dermatological uses of hdIVIg have been limited to either uncontrolled trials or anecdotal case reports, except for a single controlled trial of hdIVIg as adjunctive therapy in patients with dermatomyositis, which documented a significant benefit. Further trials in dermatomyositis should be established to confirm these data and to clarify the dose and frequency of therapy required for patients with dermatomyositis. When using hdIVIg, liaison between the dermatologist and the immunologist is helpful because it allows the use of both the nursing and the medical expertise of an existing immunotherapy unit. If appropriate, the patient may be entered into an hdIVIg home therapy training program, such as the one that exists for primary immunodeficiency and some neurologic indications, with clear benefits in quality of life and inpatient costs.

Adjuvants, Immunologic↗

Extracorporeal photopheresis in Sézary syndrome. No significant effect in the survival of 44 patients with a peripheral blood T-cell clone.

BACKGROUND: Several retrospective studies have claimed that extracorporeal photopheresis (ECP) prolongs survival in patients with erythrodermic cutaneous T-cell lymphoma. In a retrospective study of 44 patients with Sézary syndrome, we compared survival in patients treated with ECP with that of patients treated conventionally at the same institute. All patients had genotypic evidence of a peripheral blood T-cell clone. OBSERVATIONS: Twenty-nine patients received ECP (group 1); 15 patients did not receive ECP, 8 patients when ECP was available (group 2) and 7 before ECP was available (group 3). Forty-three of 44 patients received other conventional treatments. Median survival from diagnosis of Sézary syndrome was 39 months in group 1, 22 months in group 2, and 27.5 months in group 3 (Kaplan-Meier analysis). Cox regression analysis showed no significant difference between the 3 groups after correcting for age, sex, and initial Sézary cell count (hazard ratio, 0.56; 95% confidence interval, 0.26-1.17; P = .12). CONCLUSIONS: This study does not support the contention that ECP prolongs survival in patients with Sézary syndrome. The median survival in the ECP-treated group is considerably less than that reported in other published series, possibly because genotypic evidence of clonality in the peripheral blood was required for inclusion in this study. We believe that a randomized trial comparing ECP with standard chemotherapy is urgently needed.

Age Factors↗

Introducing quality assurance to health service delivery--some approaches from South Africa, Ghana and Kenya.

The following report combines the contributions of four health care professionals engaged in quality improvement activities in three different areas of Africa--Ghana in West Africa, Kenya in the East, and the Republic of South Africa. These reports provide current and vivid accounts of the impact of quality assurance and quality improvement activities on areas of change in each country's rapidly expanding health care system.

Delivery of Health Care↗

Lymphomatoid papulosis in association with mycosis fungoides: a study of 15 cases.

We report clinical findings in 15 patients with lymphomatoid papulosis (LyP) associated with mycosis fungoides (MF). LyP either preceded (n = 4), followed (n = 5) or occurred concurrently with the MF lesions (n = 6). Twenty-eight LyP lesions were classified histologically and analysed further with immunostaining for CD3 and CD30. Five biopsies contained a predominance of type A cells, six biopsies contained a predominance of type B cells. and six were mixed (A + B). However, 11 biopsies contained a population of atypical mononuclear cells with large hyperchromatic nuclei that we have termed indeterminate cells. These cells contained a thin rim of eosinophilic cytoplasm and showed strong CD30 but absent, faint or normal CD3 staining. In seven biopsies from five separate patients these cells represented the predominant cell type and we have termed this the pleomorphic variant of LyP. Analysis of T-cell receptor genes using Southern blot analysis and polymerase chain reaction/single strand conformational polymorphism analysis identified a T-cell clone in six of 16 LyP lesions and nine of 16 MF lesions. In the three patients who had clones in both types of skin lesions, the clones were identical. Only two of 10 blood samples, both of which were from the same patient, had a T-cell clone and none of two lymph nodes showed evidence of a clonal population. To date all patients are alive with a median follow-up of 15 years from the onset of the first lesion. One patient has developed Lin anaplastic large cell lymphoma of the nasopharynx. These data augment the current literature on the association of LyP and MF and suggest that the

Adolescent↗

Clinical and pathological heterogeneity in cutaneous gamma-delta T-cell lymphoma: a report of three cases and a review of the literature.

Cutaneous gamma-delta (gamma delta) T-cell lymphoma is rare. Eleven cases have been reported to date including four cases of mycosis fungoides (MF), two of pagetoid reticulosis and five of pleomorphic cutaneous T-cell lymphoma (CTCL). We report three further cases of cutaneous gamma delta T-cell lymphoma; one of MF, one of a pleomorphic CTCL and one of a subcutaneous T-cell lymphoma. Combined data suggest that although cutaneous gamma delta T-cell lymphomas do not appear to comprise a single clinicopathological entity, they may be associated with aggressive clinical behaviour and a poor prognosis.

Adult↗