PubMed Health⌕ Search

Biomedical subjects

R Russell-Jones

Publications and source records attributed to R Russell-Jones.

At least 19 recordsLinked to original sources
PubMed2003

Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathological, immunophenotypic and molecular analysis of six patients.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma of the skin. In the World Health Organization classification of T-cell and natural killer cell lymphoma it is listed as an example of extranodal lymphoma. In practice, however, it is most likely to present to a dermatologist. OBJECTIVES: To describe the clinicopathological, immunophenotypic and molecular features of six U.K. patients with SPTCL. METHODS: The clinical, histological and immunophenotypic features were reviewed. T-cell receptor (TCR) gene analysis was performed on blood and tissue samples using polymerase chain reaction/single-strand conformational polymorphism analysis of the TCR-gamma gene using consensus primers. In situ hybridization was performed on lesional skin to detect mRNA for Epstein-Barr virus (EBV). RESULTS: All patients presented with subcutaneous nodules, plaques or ulceration, and three had systemic symptoms. All biopsies exhibited an infiltrate of medium to large pleomorphic cells involving the subcutis with characteristic rimming of fat spaces. Five showed areas of necrosis, but only one showed marked cytophagia. In three cases the neoplastic cells did not express TCR-beta. One was strongly p53 positive, and the other two were CD56 positive. Both these patients showed epidermal involvement with lichenoid changes histologically, and both developed the haemophagocytic syndrome. The other three cases were TCR-beta positive, CD8 positive and CD56 negative. All cases were positive with pan T-cell markers and also for the cytotoxic granule protein T-cell intracellular antigen-1 and granzyme B. All cases were EBV negative both by immunostaining (latent membrane protein-1) and by in situ hybridization (EBV-encoded mRNA). TCR gene analysis revealed a T-cell clone in four of five cases; two of these patients had an identical T-cell clone in the peripheral blood. The median survival was 16 months. However, two of the three TCR-beta-negative patients have died, whereas none of the TCR-beta-positive patients has died. CONCLUSIONS: This is the first series of SPTCL patients to be reported in the U.K. and the data support the view that there are two subsets of SPTCL: those derived from gammadelta T cells which carry a poor prognosis, and are usually CD56 positive, and a more indolent group derived from alphabeta T cells.

Adult↗
PubMed2003

Blastic natural killer cell and extranodal natural killer cell-like T-cell lymphoma presenting in the skin: report of six cases from the UK.

BACKGROUND: Some lymphomas express natural killer (NK)-cell markers such as the neural cell adhesion molecule, which is recognized by the CD56 antibody. These lymphomas may present in the skin, but do not represent a homogeneous group. The new World Health Organization classification of lymphoma/leukaemia recognizes several types of NK/T-cell neoplasm, including blastic NK-cell lymphoma, which characteristically presents with cutaneous lesions. OBJECTIVES: To describe the clinical, pathological and molecular features in six cases of CD56+ lymphoma with cutaneous presentation. METHODS: The clinical, histopathological and immunophenotypic features of six patients were reviewed. In addition, in situ hybridization (ISH) to identify Epstein-Barr virus (EBV) mRNA, and polymerase chain reaction analysis to identify the presence of a clonal population of T cells or B cells were performed on lesional skin. RESULTS: All patients presented with widespread nodules and plaques, which in five cases were a characteristic purple colour. Four patients developed disseminated disease, three with neurological involvement. These four patients died between 14 and 46 months following diagnosis (median 30 months). In four of six cases the histopathological and immunohistological features were in keeping with a blastic NK-cell lymphoma. No clonal immunoglobulin heavy chain (IgH) or T-cell receptor (TCR) gene rearrangement was detected in the four cases consistent with an origin from NK cells. A further case fitted the criteria for an extranodal NK/T-cell lymphoma of nasal type and was also the only case to show evidence of EBV mRNA by ISH. A clonal T-cell population was identified in the final case. This patient also exhibited molecular evidence of a clonal B-cell population and a t(14;18) translocation confirmed by sequence analysis. CONCLUSIONS: Our data confirm that NK-cell lymphomas presenting in the skin are a heterogeneous group, and that in the U.K., blastic NK-cell lymphoma is more common than extranodal NK/T-cell lymphoma of nasal type. These lymphomas pursue an aggressive course, with rapid development of disseminated disease, and resistance to chemotherapy. Detailed immunophenotyping is needed to distinguish the different types. Our molecular data indicate that blastic NK-cell lymphoma cases lack clonal TCR/IgH gene rearrangements consistent with an NK-cell origin. Our ISH findings indicate that EBV plays a pathogenetic role only in extranodal NK/T-cell lymphoma of nasal type.

Adult↗
PubMed2003

Giant condyloma of Buschke-Loewenstein in association with erythroderma.

The Buschke-Loewenstein tumour is regarded as a type of verrucous carcinoma occurring on anogenital mucosal surfaces. The tumour is locally invasive but displays a benign cytology and rarely metastasizes. It is associated with human papillomavirus types 6 and 11. We describe a case of Buschke-Loewenstein tumour occurring in a 61-year-old man which behaved in a locally aggressive manner and was associated with human papillomavirus type 16 and erythroderma which proved resistant to treatment. The patient refused surgery and therapy with interferon alpha was ineffective. Chemotherapy with systemic cisplatin and 5-fluorouracil produced a partial response before the patient succumbed from gram-negative septicaemia.

Anti-Bacterial Agents↗
PubMed2003

Tea tree oil dermatitis associated with linear IgA disease.

Tea tree oil dermatitis is an increasingly common finding, reflecting the strong demand for natural remedies and aromatic substances. Linear immunoglobulin A (IgA) disease is a rare acquired subepidermal blistering disorder, characterized by basement membrane zone IgA deposition. We describe a patient in whom linear IgA disease appears to have been precipitated by a contact reaction to tea tree oil.

Adolescent↗
PubMed2002

Serum S100 concentrations are not useful in predicting micrometastatic disease in cutaneous malignant melanoma.

BACKGROUND: S100 protein is an acidic calcium binding protein that is expressed by melanoma cells. Elevated serum values of S100 have been described in metastatic disease and it has been suggested that it may be used as an adjunct to staging and monitoring of treatment. Micrometastatic disease in the sentinel lymph node can be demonstrated by sentinel node biopsy (SNB) and the sentinel node status is known to be the most important predictor of relapse. OBJECTIVES: To determine whether serum S100 concentrations could predict the presence of micrometastatic disease. METHODS: Thirty-one patients with primary cutaneous melanoma > 1 mm were recruited from referrals to the Melanoma clinic. All patients had serum S100 concentrations evaluated prior to undergoing SNB. Serum S100 concentrations were established using an immunoluminometric method. Sentinel nodes were identified using a dual technique with both radiolabelled colloid (residual from preoperative lymphoscintigraphy) and blue dye according to the MD Anderson Cancer Center protocol. Results Nine of these 31 patients had evidence of micrometastatic disease on SNB. The mean serum S100 concentration of those with positive SNBs was 0.027 microg L-1 compared with 0.045 microg x L(-1) in patients with negative SNBs (normal < 0.14 microg x L(-1)). No patient in the study demonstrated raised concentrations of serum S100. CONCLUSIONS: We conclude that serum S100 concentrations do not predict the presence of micrometastatic melanoma in sentinel nodes in primary cutaneous melanoma.

Biomarkers, Tumor↗
PubMed2002

Lymphocytic infiltrates as a presenting feature of Sweet's syndrome with myelodysplasia and response to cyclophosphamide.

Sweet's syndrome has a well-recognized association with malignancies, around half of which have been acute myelogenous leukaemia. There are also numerous reports of Sweet's syndrome in association with myelodysplasia. We report two patients with Sweet's syndrome in whom the classical histological appearances were preceded by dermal lymphocytic infiltrates. A literature search using PubMed indicates that this phenomenon has not been previously reported. The cases demonstrate the chronicity of Sweet's lesions in association with haematological disease and the need for repeat biopsies to make the diagnosis. We also describe successful treatment with cyclophosphamide, which adds to the list of second-line drugs that may be used in Sweet's syndrome.

Aged↗
PubMed2002

Response of livedoid vasculitis to intravenous immunoglobulin.

Livedoid vasculitis is a chronic condition characterized by recurrent painful ulceration of the lower limbs, which heals to leave atrophie blanche surrounded by hyperpigmentation and telangiectasia. We report two patients with livedoid vasculitis who, after failure of conventional therapies, responded to intravenous immunoglobulin (IVIg). There was healing of areas of active ulceration and improvement of erythema, swelling and pain. IVIg has been used successfully to treat a variety of vasculitic disorders and appears to be well tolerated. We suggest that this treatment is offered to patients who have livedoid vasculitis that is unresponsive to other therapies.

Adult↗
PubMed2002

Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sézary syndrome and mycosis fungoides.

BACKGROUND: Data on genome-wide surveys for chromosome aberrations in primary cutaneous T-cell lymphoma (CTCL) are limited. OBJECTIVE: To investigate genetic aberrations in CTCL. METHODS: We analysed 18 cases of Sézary syndrome (SS) and 16 cases of mycosis fungoides (MF) by comparative genomic hybridization (CGH) analysis, and correlated findings with the results of additional conventional cytogenetics, fluorescent in situ hybridization (FISH) and allelotyping studies. RESULTS: CGH analysis showed chromosome imbalances (CIs) in 19 of 34 CTCL cases (56%). The mean +/- SD number of CIs per sample was 1.8 +/- 2.4, with losses (1.2 +/- 2.0) slightly more frequent than gains (0.6 +/- 1.0). The most frequent losses involved chromosomes 1p (38%), 17p (21%), 10q/10 (15%) and 19 (15%), with minimal regions of deletion at 1p31p36 and 10q26. The commonly detected chromosomal gains involved 4/4q (18%), 18 (15%) and 17q/17 (12%). Both SS and late stages of MF showed a similar pattern of CIs, but no chromosomal changes were found in three patients with T1 stage MF. Of the 18 SS cases also analysed by cytogenetics, seven showed clonal chromosome abnormalities (39%). Five cases had structural aberrations affecting chromosomes 10 and 17, four demonstrated rearrangement of 1p and three revealed an abnormality of either 6q or 14q consistent with CGH findings. FISH analysis showed chromosome 1p and 17q rearrangements in five of 15 SS cases, and chromosome 10 abnormalities in four SS cases consistent with both the G-banded karyotype and the CGH results. In addition, allelotyping analysis of 33 MF patients using chromosome 1 markers suggested minimal regions of deletion at D1S228 (1p36), D1S2766 (1p22) and D1S397 (1q25). CONCLUSIONS: These findings provide a comprehensive assessment of genetic abnormalities in CTCL and a rational approach for further studies.

Chromosome Aberrations↗
PubMed2002

Childhood granulomatous periorificial dermatitis in an Asian girl--a variant of sarcoid?

Childhood granulomatous periorificial dermatitis (CGPD) is a benign, facial eruption which has previously been reported in prepubertal, Afro-Caribbean children. It is a condition of unknown aetiology, characterized by a monomorphic, papular eruption around the mouth, nose and eyes. Typically the eruption persists for several months but resolves without scarring. The condition can usually be distinguished from sarcoidosis on clinical and histological grounds. To our knowledge, this is the first case of CGPD occurring in an Asian child with sarcoidal histology and is unusual in its persistence for 4 years. We suggest that this case of CGPD may well be a variant of sarcoid.

Asia↗
PubMed2001

Diagnostic and prognostic importance of T-cell receptor gene analysis in patients with Sézary syndrome.

BACKGROUND: Sézary syndrome (SS) is characterized by erythroderma, peripheral lymphadenopathy, and circulating Sézary cells and is clinically heterogeneous. METHODS: T-cell receptor (TCR) gene analysis was performed using DNA extracted from peripheral blood mononuclear cells from 74 patients, and the results were correlated with a variety of other diagnostic parameters and patient outcomes. RESULTS: Two groups were identified: 66 patients with clonal TCR gene rearrangement (clonal patients) detected with Southern blot analysis and/or polymerase chain reaction/single-strand conformational polymorphism analysis and 8 patients with no clonal rearrangement detected (nonclonal patients) using either technique. Clonal patients were compared with nonclonal patients. The following median blood parameters were significantly greater in the clonal group: total white cell count (13.7 10(9)/L vs. 9.6 10(9)/L), lymphocyte count (4.9 10(9)/L vs. 2.2 10(9)/L), absolute Sézary count (3.22 10(9)/L vs. 0.99 10(9)/L), CD4 count (3.17 10(9)/L vs. 1.36 10(9)/L), and CD4:CD8 ratio (15.86 vs. 3.21). An expanded population of T-cells of a specific TCR variable beta subset was detected in 7 of 36 clonal patients and in 1 of 4 nonclonal patients. Cytogenetic analysis of peripheral blood from 1 nonclonal patient and 6 clonal patients was normal. The median survival from the time of diagnosis was 45 months in the clonal group, and 40 of 49 deaths were cutaneous T-cell lymphoma (CTCL)-related, whereas 3 deaths in the nonclonal group were unrelated to CTCL (P < 0.01; log-rank test). Multivariate proportional hazards analysis showed that the absolute Sézary count and lymph node status were independent prognostic variables (P = 0.016 and P = 0.036, respectively). CONCLUSIONS: TCR gene analysis defines a distinct clinicopathologic group of patients with SS. Clonal patients have a poor prognosis and are likely to die from leukemia/lymphoma, whereas nonclonal patients may have a reactive, inflammatory T-cell disorder. The authors suggest that the definitive diagnostic criteria for patients with SS should include the presence of a clonal TCR gene rearrangement.

Adult↗
PubMed2001

Extracorporeal photopheresis in Sézary syndrome: hematologic parameters as predictors of response.

Data were analyzed from 23 patients with Sézary syndrome (defined by erythroderma, more than 10% circulating atypical mononuclear cells, and peripheral blood T-cell clone) undergoing monthly extracorporeal photopheresis as the sole therapy for up to 1 year. The cohort showed a significant reduction of skin scores during treatment (P =.001). Thirteen patients (57%) achieved a reduction in skin score greater than 25% from baseline at 3, 6, 9, or 12 months (responders). Reduction in skin score correlated with reduction in the Sézary cell count as a percentage of total white cell count (P =.03). Responders and nonresponders were compared. None of the measured parameters was significantly different between the 2 groups. It was assessed whether any of the baseline parameters predicted outcome. A higher baseline lymphocyte count was significantly associated with a decrease in skin score at 6 months (P <.05). A higher baseline Sézary cell count as a percentage of total white cell count predicted a subject was more likely to be a responder after 6 months of treatment (P =.021). No other parameters predicted responder status. These data show that the modest falls in CD4, CD8, and Sézary cell counts were seen in all patients and might have resulted from lymphocyte apoptosis. This mechanism could explain the more favorable response seen in patients with higher percentages of Sézary cells in the peripheral blood. Alternatively, minimum tumor burden might be required for the induction of a cytotoxic response. Analysis of tumor-specific cytotoxic T cells is needed to investigate these possibilities further.

Adult↗
PubMed2001

Comparison of positron emission tomography scanning and sentinel node biopsy in the detection of micrometastases of primary cutaneous malignant melanoma.

PURPOSE: Sentinel node biopsy (SNB) is a surgical technique for detecting micrometastatic disease in the regional draining nodes. 2-fluorine-18-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) scanning is an imaging technique that can detect clinically undetectable metastases. This prospective study was undertaken to compare the sensitivity of FDG-PET scanning with SNB in the detection of micromatastatic malignant melanoma. PATIENTS AND METHODS: Fifty consecutive patients (23 women, 27 men; mean age, 53 years) with primary melanoma >1 mm thick or lymphatic invasion were recruited (mean, 2.41 mm). Primary lesions had been narrowly excised (<1 cm). Patients underwent PET scanning followed by SNB, using a dual technique. Preoperative lymphoscintigraphy was used to identify the draining basin. Lymph nodes were examined histologically and immunostained for S100 and HMB 45. RESULTS: The sentinel node (SN) was identified in all patients. Fourteen patients (28%) had positive SNBs, including eight patients with melanoma <1.5 mm thick. In none of these 14 patients did PET scans identify metastatic disease in the SN or draining basin. In seven patients, the PET scans were positive in other locations, and in four cases, this was suspicious of metastatic disease. However, no patient has developed recurrent melanoma (mean follow-up, 15 months). All patients with positive SNBs underwent therapeutic lymph node dissection. Further lymph node involvement was found in two patients (primary lesions, 1.3 mm and 3.5 mm thick). CONCLUSION: This study demonstrates the limitations of FDG-PET scanning in staging patients with primary melanoma. SNB is the only reliable method for identifying micrometastatic disease in the regional draining node.

Adult↗
PubMed2001

Prognostic significance of tumor burden in the blood of patients with erythrodermic primary cutaneous T-cell lymphoma.

Erythrodermic cutaneous T-cell lymphoma (CTCL) includes patients with erythrodermic mycosis fungoides who may or may not exhibit blood involvement and Sézary syndrome and in whom hematological involvement is, by definition, present at diagnosis. These patients were stratified into 5 hematologic stages (H0-H4) by measuring blood tumor burden, and these data were correlated with survival. The study identified 57 patients: 3 had no evidence of hematologic involvement (H0), 8 had a peripheral blood T-cell clone detected by polymerase chain reaction (PCR) analysis of the T-cell receptor gene and less than 5% Sézary cells on peripheral blood smear (H1), and 14 had either a T-cell clone detected by Southern blot analysis or PCR positivity with more than 5% circulating Sézary cells (H2). Twenty-four patients had absolute Sézary counts of more than 1 x 10(9) cells per liter (H3), and 8 patients had counts in excess of 10 x 10(9) cells per liter (H4). The disease-specific death rate was higher with increasing hematologic stage, after correcting for age at diagnosis. A univariate analysis of 30 patients with defined lymph node stage found hematologic stage (P =.045) and lymph node stage (P =.013) but not age (P =.136) to be poor prognostic indicators of survival. Multivariate analysis identified only lymph node stage to be prognostically important, although likelihood ratio tests indicated that hematologic stage provides additional information (P =.035). Increasing tumor burden in blood and lymph nodes of patients with erythrodermic CTCL was associated with a worse prognosis. The data imply that a hematologic staging system could complement existing tumor-node-metastasis staging criteria in erythrodermic CTCL.

Adult↗
PubMed2001

Allelotyping in mycosis fungoides and Sézary syndrome: common regions of allelic loss identified on 9p, 10q, and 17p.

Allelotyping studies have been extensively used in a wide variety of malignancies to define chromosomal regions of allelic loss and sites of putative tumor suppressor genes; however, until now this technique has not been used in cutaneous lymphoma. We have analyzed 51 samples from patients with mycosis fungoides and 15 with Sézary syndrome using methods to detect loss of heterozygosity. Micro satellite markers were selected on 15 chromosomal arms because of their proximity to either known tumor suppressor genes or chromosomal abnormalities identified in previous cytogenetic studies in cutaneous lymphoma. Allelic loss was present in 45% of patients with mycosis fungoides and 67% with Sézary syndrome. Loss of heterozygosity was found in over 10% of patients with mycosis fungoides on 9p, 10q, 1p, and 17p and was present in 37% with early stage (T1 and T2) and 57% with advanced disease (T3 and T4). Allelic loss on 1p and 9p were found in all stages of mycosis fungoides, whereas losses on 17p and 10q were limited to advanced disease. In Sézary syndrome high rates of loss of heterozygosity were detected on 9p (46%) and 17p (42%) with lower rates on 2p (12%), 6q (7%), and 10q (12%). There was no significant difference in the age at diagnosis or number of treatments received by those with loss of heterozygosity and those without, suggesting that increasing age and multiple treatments do not predispose to allelic loss. These results provide the basis for further studies defining more accurately chromosomal regions of deletions and candidate tumor suppressor genes involved in mycosis fungoides and Sézary syndrome.

Chromosomes, Human, Pair 10↗
PubMed2001

Molecular analysis of the immunoglobulin heavy chain gene in the diagnosis of primary cutaneous B cell lymphoma.

The diagnosis of primary cutaneous B cell lymphoma can be difficult on the basis of histologic and immunophenotypic features alone. Previous polymerase chain reaction studies for detection of a clonal population in nodal B cell lymphomas have employed different primer pairs with detection sensitivities varying between 34% and 94% but there have been no comprehensive studies of primary cutaneous B cell lymphoma. We compared the sensitivity of different sets of consensus primers to amplify the CDR3 VDJ region of the immunoglobulin heavy chain gene in combination with an immunoglobulin heavy chain joining region consensus primer to detect a monoclonal population in 39 cases of primary cutaneous B cell lymphoma. Radiolabeled products were analyzed with denaturing 6% polyacrylamide gel electrophoresis. Sequence analysis was used to confirm amplification of clonal immunoglobulin heavy chain gene rearrangements and to establish whether somatic hypermutation can interfere with primer binding. Clonal immunoglobulin heavy chain gene rearrangements were demonstrated in 79% of cases (74% with leader sequences, 64% with FR1, and 45% with FR3 primers). Somatic hypermutation at primer binding sites was confirmed in cases where a false negative result was obtained with the FR3 primer. Although monoplex polymerase chain reaction amplification using the leader sequence primers is the most sensitive method for detecting a clonal population, six primers are required in six different reactions. Our findings suggest initial analysis with the FR3 primer and subsequent analysis using leader sequences in negative cases. Our data indicate that the FR3 consensus primer alone is not sufficient for a comprehensive analysis of primary cutaneous B cell lymphoma.

Base Sequence↗