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R Russell-Jones

Publications and source records attributed to R Russell-Jones.

At least 73 records · Page 4Linked to original sources
PubMed2000

Systemic Hodgkin's lymphoma in a patient with Sézary syndrome.

We report a case of a 71-year-old male with Sézary syndrome diagnosed in 1996 who subsequently developed systemic Hodgkin's lymphoma. His only past treatment was bath psoralen plus ultraviolet A. He has since been treated with multiagent chemotherapy (ChlVPP/PABLOE) which induced a remission in his Hodgkin's disease. Eighteen months later he remains in remission from Hodgkin's disease but the Sézary syndrome remains active. He has also developed a squamous cell carcinoma on the upper lip. Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by a malignant proliferation of CD4-positive cells in the skin and peripheral circulation. The CD4 count may be markedly elevated but this results from expansion of a neoplastic T-cell clone and there is a relative lymphopenia of normal T cells leading to a degree of immunoparesis. Immunosuppression is known to be associated with an increased rate of malignancies and this may account for the occurrence of Hodgkin's disease and squamous cell carcinoma in this patient with Sézary syndrome.

Aged↗
PubMed2000

Rituximab in cutaneous B-cell lymphoma: a report of two cases.

We report two patients with primary cutaneous B-cell lymphoma who were treated with rituximab, a new anti-CD20 monoclonal antibody. The first patient, who had a diffuse large B-cell lymphoma of the lower leg, achieved an 85% improvement. The second patient, who had a primary cutaneous B-cell lymphoma, which had undergone high-grade transformation and systemic spread, achieved a minor response of approximately 30%. Both patients subsequently relapsed. The first patient achieved complete clearance with a second course of rituximab given with systemic chemotherapy, but again relapsed. Treatment with rituximab has been reported to produce response rates of 48% in relapsed systemic low-grade or follicular lymphoma, but there are no previous reports of the use of rituximab in primary cutaneous B-cell lymphoma.

Adult↗
PubMed2000

Multiple cutaneous immunocytoma with secondary anetoderma: a report of two cases.

We describe two men with multiple erythematous dermal nodules which were clinically and histologically consistent with a diagnosis of primary cutaneous immunocytoma. Both patients exhibited the very unusual feature of secondary anetoderma occurring in spontaneously resolving lesions. There is one previous report of anetoderma in association with a plasmacytoma. The pathogenesis remains unknown but release of cytokines such as interleukin-6 may be implicated.

Adult↗
PubMed2000

Eosinophilic spongiosis in association with bullous pemphigoid and chronic lymphocytic leukaemia.

We report the onset of an unusual blistering eruption following a diagnosis of B-cell chronic lymphocytic leukaemia (CLL). The histology consistently showed eosinophilic spongiosis, but the diagnosis of bullous pemphigoid was only confirmed after 13 years by repeated immunofluorescence studies. The occurrence of subepidermal blistering diseases in association with B-cell lymphoproliferative disorders is rare; a recent study showed that the majority of these cases are epidermolysis bullosa acquisita, confirmed by immunological studies. Only two cases of immunofluorescence-proven bullous pemphigoid in association with CLL have been previously reported.

Aged↗
PubMed2000

CD8-positive juvenile onset mycosis fungoides: an immunohistochemical and genotypic analysis of six cases.

BACKGROUND: Childhood cases of cytotoxic T-cell lymphoma have not been well described. OBJECTIVES: We have undertaken an immunohistochemical and genotypic analysis of patients presenting with juvenile onset mycosis fungoides (MF). PATIENTS/METHODS: Of 10 patients presenting over a 3-year period, six exhibited a CD8-positive phenotype. These six patients were also CD2, CD3 and TIA1 positive, but CD56 negative. Apart from the cytotoxic phenotype, these patients had clinicopathological features that were indistinguishable from ordinary cases of MF, with slowly evolving patches and plaques. Three patients were staged as 1A and three as 1B, with no evidence of nodal or systemic disease. RESULTS: Patients responded well to conventional therapy, with no evidence of disease progression after 3 years follow-up. Epidermotropism was a prominent feature in four of the six cytotoxic cases. In two patients with an equivocal histology the diagnosis was confirmed by the finding of a clonal population, using polymerase chain reaction/single strand conformational polymorphism analysis of the T-cell receptor gamma gene in lesional skin. The same technique revealed that all blood samples analysed were polyclonal. CONCLUSIONS: These data show that cytotoxic T-cell lymphoma can pursue an indolent course and that cases of CD8-positive MF may be over-represented in childhood.

Adolescent↗
PubMed2000

Interferon-alpha therapy for melanoma.

Although surgery may be curative in early malignant melanoma, its effect on survival lessens with each succeeding stage of the disease. A wide variety of immunological strategies have therefore been used to improve the prognosis of patients with malignant melanoma, but adjuvant therapy with interferon (IFN)-alpha is the only treatment to show a therapeutic benefit in randomized controlled studies. The current data indicates that where IFN-alpha is used at low dose, its main effect is on disease-free survival, whereas high-dose regimens may improve overall survival as well. This paper will review the published data on IFN-alpha therapy in patients with intermediate and high-risk melanoma and explore future avenues for managing patients with this difficult disease.

Antineoplastic Agents↗
PubMed2000

Detection of a peripheral blood T cell clone is an independent prognostic marker in mycosis fungoides.

UNLABELLED: T cell receptor gene analysis is a sensitive method for assessment of peripheral blood involvement in mycosis fungoides. This study uses polymerase chain reaction/single-strand conformational polymorphism (PCR/SSCP) analysis of the T cell receptor gamma gene and relates the results to skin stage and outcome in mycosis fungoides. Seventy-five peripheral blood samples from 66 patients were obtained from 1990 onwards and subjected to PCR/SSCP. Both Southern blot analysis and PCR/SSCP analysis were performed on 63 samples from 56 patients. Fourteen patients had T1 disease (12 IA, two IIA), 20 T2 (14 IB, five IIA, one IVA), 29 T3 (24 IIB, two IVA, three IVB, two patients tested at both T2 and T3), and five T4 (all III). The percentage of positive samples was higher with PCR/SSCP than with Southern blot analysis (29 of 63 vs eight of 63 samples, p < 0.001), and the percentage of positive samples increased with each stage (21% at T1, 35% at T2, 58% at T3, and 71% at T4). Proportional hazards analysis corrected for age, skin, and lymph node stage showed that the presence of a peripheral blood clone is associated with a worse outcome (p = 0.03, CI 1.1-6.03). These results indicate that the presence of a peripheral blood clone is an independent prognostic variable in patients with mycosis fungoides after correcting for age, skin, and lymph node stage, and that peripheral blood involvement is present in a large proportion of patients with early stage mycosis fungoides. KEYWORDS: polymerase chain reaction/single-strand conformational polymorphism/T cell receptor gene rearrangement. J Invest Dermatol 114:117-121, 2000

Biomarkers↗
PubMed2000

The value of positron emission tomography scanning in the detection of subclinical metastatic melanoma.

We have undertaken a retrospective analysis of all positron emission tomography (PET) scans carried out at St Thomas' Hospital, London, since 1994 to establish the sensitivity and specificity of this radiologic technique in cutaneous malignant melanoma. In particular, we have identified those patients with primary cutaneous malignant melanoma in whom PET scanning revealed in-transit or regional spread to nodes and those patients with known regional spread in whom PET scanning revealed distant metastases. We defined our false-negative results as a negative scan result with positive histology or subsequent clinical progression of disease. False-positive results were defined as a suspect scan with negative histology or no subsequent progression of disease. PET scanning had an overall sensitivity of 78% and specificity of 87%; however, subset analysis (M. D. Anderson staging system) showed a sensitivity of 50% for stage I disease (34 patients and 35 scans) and 33% for stage II disease (9 patients and 9 scans) with specificities of 87% and 100%, respectively. For stage III disease (16 patients and 17 scans), PET showed a sensitivity of 93% and specificity of 50%. Overall, 35% of patients with true-positive scans had their disease restaged. We can conclude therefore that PET is valuable as a staging procedure in patients with known regional spread but is suboptimal in the prediction of outcome in stage I or stage II disease.

Female↗
PubMed1999

Secondary malignant neoplasms in 71 patients with Sézary syndrome.

BACKGROUND: Sézary syndrome (SS) is characterized by a malignant proliferation of CD4+ve T cells, which may result in a degree of immunoparesis. Immunosuppression is associated with an increased incidence of internal malignant neoplasms and a high rate of nonmelanoma skin cancer, particularly squamous cell carcinoma. Therefore, we reviewed the incidence of secondary malignant neoplasms in patients with SS. OBSERVATIONS: Of 71 patients with SS, 16 (23%) developed 19 secondary and tertiary malignant neoplasms. These malignant neoplasms included 8 cutaneous squamous cell carcinomas, 2 squamous cell carcinomas of the oral mucosa, and 9 other internal malignant neoplasms. The incidence of internal malignant neoplasms was twice that reported in patients of similar age treated for Hodgkin disease (P = .02). Furthermore, the incidence of cutaneous squamous cell carcinoma in the cohort was 42 times that observed in a study conducted in England of an age-matched population (1657 per 1 x 10(5) vs 39 per 1 x 10(5) person-years [95% confidence interval, 626-2856]). CONCLUSIONS: A number of therapeutic modalities for SS are known to be carcinogenic. We compared the different therapeutic modalities received by our patients and found no significant difference between the total cohort of patients with SS and the patients who developed secondary malignant neoplasms. These data indicate that the high incidence of secondary malignant neoplasms in patients with SS is due, at least in part, to the disease itself. The clonal proliferation of CD4+ve T cells and the relative lymphopenia (compared with a healthy population) of nonneoplastic T cells may result in compromised immunosurveillance, so that early neoplasia, whether arising spontaneously or as a result of therapy, are not dealt with appropriately.

Adult↗
PubMed1999

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↗
PubMed1999

Striae formation in two HIV-positive persons receiving protease inhibitors.

Steroidal side effects such as "buffalo hump" and visceral abdominal fat accumulation have been reported in association with protease inhibitors. We report two cases of severe disfiguring striae formation in patients with HIV who recently started indinavir therapy. These changes occurred within 3 months of starting treatment.

Drug Therapy, Combination↗
PubMed1999

Extracorporeal photopheresis (ECP) in the treatment of chronic graft-versus-host disease (GVHD)

The aim of our study was to assess the efficacy of extracorporeal photopheresis (ECP) in chronic graft-versus-host disease (GVHD). Eleven patients with chronic cutaneous GVHD were studied. Four had mucosal involvement and five had pulmonary involvement. All had failed to improve on first- and second-line therapy. Three patients received ECP alone; the remainder continued to receive steroids and/or immunosuppressive therapy. Patients received ECP twice monthly for 4 months and then once monthly for 3 months. They were evaluated by serial skin scores, mucosal and skin photography, pulmonary function tests, biochemical and haematological parameters. Nine patients showed objective evidence of cutaneous improvement with a mean reduction in skin score of 48% overall. In the 10th patient, skin scores and oral involvement improved on twice monthly ECP but deteriorated when reduced to once monthly. The final patient died from renal failure secondary to cyclosporin toxicity. Two out of five patients with lung involvement showed a mild improvement in pulmonary function tests. Liver function tests were abnormal in five patients; they improved in one and deteriorated in three. All patients receiving concomitant immunosuppressive/steroid therapy were able to reduce drug dosages by trial completion. Our results indicate that ECP can benefit patients with cutaneous and mucosal chronic GVHD who have failed on first- and second-line therapies. The effect on the systemic manifestations of GVHD is less consistent.

Adolescent↗
PubMed1999

Melanoma antigen-encoding gene expression in melanocytic naevi and cutaneous malignant melanomas.

Melanoma antigen-encoding (MAGE) genes encode peptides which are expressed by a proportion of malignant melanomas (MMs) and can be recognized in vitro by autologous cytolytic T lymphocytes in a human leucocyte-associated antigen (HLA)-restricted manner. Although expression of MAGE genes has been studied in cutaneous MMs, little is known about their expression in melanocytic naevi. We studied MAGE 1, 2, 3, 4, 6 and 12 gene expression in tissue from 10 benign melanocytic naevi, 14 dysplastic melanocytic naevi, three cutaneous MMs in situ, four primary cutaneous MMs and three distant metastatic MMs. MAGE gene expression was determined with reverse transcription-polymerase chain reaction (PCR) using random hexamers to generate cDNA from total tissue homogenate RNA followed by PCR using intron-spanning, MAGE-specific primer pairs. Controls were cDNA from MAGE-expressing melanoma cell lines. Expression of HLA class 1 was used as a cDNA quality control. MAGE 2, 4, 6 and 12 gene expression was not detected in any of the lesions studied. MAGE 1 and 3 gene expression was also not detected in any of the cases of benign and dysplastic melanocytic naevi and in situ MMs. One of four primary MMs expressed the MAGE 3 gene. Two of three distant metastatic MMs also expressed the MAGE 3 gene and one of these additionally expressed the MAGE 1 gene. Thus, MAGE gene expression appears to be a late event in the evolution of MMs.

Antigens, Neoplasm↗
PubMed1999

Livedoid vasculitis: a manifestation of the antiphospholipid syndrome?

Livedoid vasculitis, otherwise known as segmental hyalinizing vasculitis or livedo reticularis with summer ulceration, is a chronic disease with lesions affecting the feet and lower legs. Early lesions show petechiae, but characteristic features are recurrent, bizarrely shaped ulcers that heal to leave hyperpigmentation and atrophie blanche. The aetiology of the disorder is unknown, but the histology shows fibrin deposition within both the wall and lumen of affected vessels. The absence of a sufficient perivascular infiltrate or leucocytoclasia argues against a vasculitis, being more in keeping with a thrombo-occlusive process. Four patients with livedoid vasculitis with ulceration are described, all of whom had associated raised anticardiolipin antibodies but no other evidence of systemic disease. We suggest that livedoid vasculitis may be a manifestation of the antiphospholipid syndrome and recommend that all patients are screened for this. We also discuss treatment options for this often resistant condition.

Adolescent↗
PubMed1999

Pilotropic mycosis fungoides presenting with multiple cysts, comedones and alopecia.

We report a 45-year-old man with mycosis fungoides (MF) who presented with multiple epidermoid cysts, comedones and alopecia and who responded to treatment with intravenous and oral cyclophosphamide. The pilotropic variant of MF, characterized histologically by follicular lesions and pilotropism without mucinosis, is uncommon and often resistant to treatment.

Alopecia↗
PubMed1999

Staphylococcal scalded skin syndrome in an adult associated with methicillin-resistant Staphylococcus aureus.

We report the first adult case of staphylococcal scalded skin syndrome (SSSS) due to methicillin-resistant Staphylococcus aureus (MRSA). This case is particularly unusual as the MRSA produced toxic shock syndrome toxin 1 and enterotoxin, but not exfoliatoxin. SSSS was originally described in neonates and is thought to result from exfoliatins which produce subcorneal splitting of the epidermis and are only produced by certain strains of S. aureus. This case reflects the range of toxins that can be associated with SSSS and the clinical manifestations of MRSA infection in adult patients.

Aged↗
PubMed1999

B-cell lymphoma associated with chronic lymphatic leukaemia: two cases with contrasting aggressive and indolent behaviour.

The term Richter's syndrome is used to describe the transformation of chronic lymphatic leukaemia (CLL) into a high-grade systemic lymphoma and is associated with a poor prognosis. We have undertaken detailed molecular studies in two patients with cutaneous B-cell lymphoma (CBCL) and CLL. Patient 1 exhibited a low-grade CBCL with different immunoglobulin gene rearrangements in blood and skin. By contrast, patient 2 showed identical gene rearrangements, confirmed by gene sequencing, and died within 4 months of presentation. The latter patient fulfilled the criteria for a diagnosis of cutaneous Richter's syndrome, whereas the former patient demonstrated the coincidence of CLL with a primary CBCL. Our results highlight the importance of gene rearrangement studies with sequencing for the accurate diagnosis of cutaneous Richter's syndrome.

Aged↗