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At least 991 records · Page 55Linked to original sources
PubMed2005

Circulating CD8+ lymphocytes, white blood cells, and survival in patients with mycosis fungoides.

BACKGROUND: There is a need for reliable, easily measurable laboratory markers that may help dermatologists to predict the course of mycosis fungoides (MF) when they first evaluate their patients. OBJECTIVES: Our objective was to identify clinical, haematological or immunological parameters as predictors of mortality in patients with MF. METHODS: We conducted a retrospective study on a prevalent cohort of 124 patients with MF hospitalized at IDI-IRCCS, Rome, Italy, from 1983 to 2001. We calculated the proportion of patients surviving (Kaplan-Meier product-limit estimates) 5 and 10 years after first hospital admission, and hazard ratios (HR) from the Cox proportional hazards model. RESULTS: Patients' survival was linked to age and staging (lower survival in older patients and in patients with staging IIB-IV). Higher numbers of white blood cells (WBC) and neutrophils, lower numbers of CD8+ lymphocytes, low haematocrit and lower levels of albumin were significantly associated with a lower survival probability. When simultaneously accounting for age and staging, CD8+ [HR = 3.02, 95% confidence interval (CI) 1.01-9.07 for CD8+ < 250 vs. > or = 600 cells microL(-1)] and WBC (HR = 2.59, 95% CI 0.96-6.96 for WBC > or = 9000 vs. < 6000 cells microL(-1)) were associated with survival. In addition, we observed an exceedingly high risk of death (HR = 12.40, 95% CI 3.11-49.43) for patients with a combination of WBC > or = 9000 and CD8+ < 600 cells microL(-1) vs. WBC < 9000 and CD8+ > or = 600 cells microL(-1)). CONCLUSIONS: The measurement of CD8+ cells and WBC in MF seems to be a promising criterion to predict survival, and possibly to support treatment decisions and inclusion of patients in randomized controlled trials.

Age Factors↗
PubMed2006

Efficacy of 8-methoxypsoralen vs. 5-methoxypsoralen plus ultraviolet A therapy in patients with mycosis fungoides.

BACKGROUND: Psoralen plus ultraviolet (UV) A (PUVA) is the standard treatment for early stage mycosis fungoides (MF). When 8-methoxypsoralen (8-MOP) is used in PUVA therapy, it often produces intolerance reactions such as nausea, vomiting and headache. OBJECTIVES: To investigate whether 5-methoxypsoralen (5-MOP) is a safe and effective alternative to 8-MOP in PUVA therapy for MF. METHODS: A retrospective database search and chart review was done to identify patients with MF who received PUVA with either 5-MOP or 8-MOP as initial monotherapy at our institution. Between 1990 and 2004, 14 patients [seven men and seven women; mean age 70 years, range 51-82; National Cancer Institute disease stages IA (n = 6) and IB (n = 8)] received 5-MOP, and 24 patients [21 men and three women; mean age 58 years, range 28-89; disease stages IA (n = 11), IB (n = 12) and IIB (n = 1)] received 8-MOP. RESULTS: Twelve of 14 patients (86%) in the 5-MOP group and 22 of 24 (92%) in the 8-MOP group had a complete response to PUVA. These two subgroups of complete responders did not differ significantly in terms of PUVA therapy duration, number of treatments or cumulative UVA dose. They also did not differ significantly in terms of relapse-free rate [8% (one of 12) vs. 23% (five of 22)] or time to relapse [17 months (range 4-31) vs. 14 months (range 4-33)]. Moreover, PUVA maintenance therapy with either 5-MOP or 8-MOP in a subset of patients [26% (nine of 34)] did not affect long-term relapse-free status either. CONCLUSIONS: 5-MOP and 8-MOP have comparable therapeutic efficacy when used in PUVA therapy for MF.

5-Methoxypsoralen↗
PubMed2006

Human T-cell lymphotropic virus type 1 provirus and phylogenetic analysis in patients with mycosis fungoides and their family relatives.

BACKGROUND: Mycosis fungoides (MF) is a cutaneous T-cell lymphoma of unknown aetiology. A pathogenic role of human T-cell lymphotropic virus type 1 (HTLV-1) has been suggested but remains controversial. To determine whether MF is linked to HTLV-1. METHODS: Blood samples were collected from 60 patients, 15 family relatives of patients with MF (MFRs), 20 healthy controls and 10 patients with HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). The presence of HTLV-1 antibodies in serum was tested by the Western blot rp21e-enhanced test. DNA was extracted from the blood with the Qiagen blood kit. We used 500 ng of DNA either in conventional HTLV-1-specific polymerase chain reaction (PCR) or in real-time PCR using primers sk43 and sk44 together with a tax-specific fluorescent probe. RESULTS: In Western blot, antibodies against three to four HTLV-1 antigens were detected in 52% of patients with MF. All of the patients with HAM/TSP were positive, while only 7% of the MFRs and none of the 20 healthy controls reacted with HTLV-1 antigens in Western blot. One of 60 patients with MF and one of 15 MFRs were positive in HTLV-1 PCR. These two PCR-positive samples which were quantified in real-time PCR showed that fewer than five in 10(6) cells were HTLV-1 infected. We succeeded in amplifying and sequencing the 5' end of the provirus from the blood of the PCR-positive MFR by seminested PCR. A positive result was also obtained in this test. Phylogenetic tree analyses revealed a high homology of this sequence with other HTLV-1 sequences from the Middle East. The above PCR-positive MFR was the brother of a PCR-negative patient with MF. CONCLUSIONS: These findings demonstrate that HTLV-1 is probably not the aetiological agent of MF. However, it may play a role in immunosuppression and in the spreading of the disease.

Adult↗
PubMed2007

Mycosis fungoides: a dermatological masquerader.

Mycosis fungoides (MF), a low-grade lymphoproliferative disorder, is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumours or erythroderma. Diagnosis of MF can be difficult due to highly variable presentations and the sometimes nonspecific nature of histological findings. Molecular biology has improved the diagnostic accuracy. Nevertheless, clinical experience is of substantial importance as MF can resemble a wide variety of skin diseases. We performed a literature review and found that MF can mimic >50 different clinical entities. We present a structured framework of clinical variations of classical, unusual and distinct forms of MF. Distinct subforms such as ichthyotic MF, adnexotropic (including syringotropic and folliculotropic) MF, MF with follicular mucinosis, granulomatous MF with granulomatous slack skin and papuloerythroderma of Ofuji are delineated in more detail.

Diagnosis, Differential↗
PubMed2006

Association of p53 Arg72Pro polymorphism and beta-catenin accumulation in mycosis fungoides.

BACKGROUND: Aberrant activation of beta-catenin contributes to the onset of a variety of tumours. There are many tumours that display beta-catenin accumulation in the absence of mutations in its gene. Recently, abnormal accumulation of wild-type beta-catenin has been associated with mutational inactivation of the p53 tumour suppressor. OBJECTIVES: To investigate the potential role of p53 and its homologue p63 in beta-catenin deregulation and to correlate this with disease outcome. METHODS: We analysed a panel of 24 samples of mycosis fungoides (MF), the most frequent manifestation of cutaneous T-cell lymphoma (CTCL), for beta-catenin, p53 and p63 protein expression by immunohistochemistry. Based on the immunostaining results for beta-catenin protein, 11 positive cases were selected for laser microdissection, genomic DNA isolation and subsequent mutation analysis of beta-catenin exon 3 and p53 exons 4-8. RESULTS: Our findings revealed overexpression of beta-catenin, p53 and p63 in 46%, 38% and 17% of cases, respectively. The number of p53-positive cases of MF was significantly higher (P < 0.05) in the beta-catenin-positive group (73%). Sequence analysis demonstrated that wild-type beta-catenin accumulation in MF is not associated with mutational inactivation of the p53 gene and, more importantly, our data provide evidence that a common polymorphic form of p53 (Arg72Pro) is significantly associated with beta-catenin overexpression (P < 0.05). No significant differences in the three genotypes were observed between the CTCL cases and the control group, demonstrating that Arg72Pro polymorphism of the p53 gene is not associated with the risk of developing cutaneous lymphomas (P > 0.05). CONCLUSIONS: We found an association of beta-catenin and p53 overexpression without detection of structural alteration in the genes, suggesting that p53 mutation is not an important mechanism for beta-catenin activation in primary CTCL. Additionally, we speculate that the p53 codon 72 polymorphism may influence negative feedback control involving beta-catenin and p53.

Adult↗
PubMed2007

Does adjuvant alpha-interferon improve outcome when combined with total skin irradiation for mycosis fungoides?

BACKGROUND: Patients with mycosis fungoides (MF) experience frequent disease recurrences following total skin electron irradiation (TSEI) and may benefit from adjuvant therapy. OBJECTIVES: To review the McGill experience with adjuvant alpha-interferon (IFN) in the treatment of MF. METHODS: From 1990 to 2000, 50 patients with MF were treated with TSEI: 31 with TSEI alone and 19 with TSEI + IFN. Median TSEI dose was 35 Gy. In the TSEI + IFN group, IFN was given subcutaneously at 3 x 10(6) units three times per week starting 2 weeks prior to start of TSEI, continued concurrently with the radiation and for an additional 12 months following TSEI. The TSEI alone group included 16 men and 15 women with a median age of 61 years (range 31-84). The TSEI + IFN group included 14 men and five women with a median age of 51 years (range 24-83). Clinical stage was IA, IB, IIA, IIB, III and IVA in 2, 9, 4, 8, 1 and 7 patients of the TSEI group and 0, 3, 3, 7, 4 and 2 patients of the TSEI + IFN group. RESULTS: Median follow up for living patients was 70 months. All patients responded to treatment. Complete response (CR) rate was 65% following TSEI and 58% following TSEI + IFN (P = 0.6). Median overall survival (OS) was 61 months following TSEI and 38 months following TSEI + IFN (P = 0.4). Acute grade II-III dermatitis was seen in all patients. Fever, chills or myalgia were seen in 32% of patients treated with TSEI + IFN. CONCLUSIONS: Concurrent IFN and TSEI is feasible, with acceptable toxicity. Even when controlling for disease stage, the addition of IFN did not appear to increase CR rate, disease-free survival or OS.

Adult↗
PubMed2007

CD30+ large cell transformation of mycosis fungoides after psoralen plus ultraviolet A photochemotherapy.

Psoralen plus ultraviolet A (PUVA) photochemotherapy is widely used for the therapy of mycosis fungoides (MF). Clinical progression of MF is often associated with an increase in the size of tumour cells known as transformation. We report two patients with CD30+ large cell transformation that appeared after low-dose PUVA therapy for MF. Clinical data, histopathology, immunohistopathology and T-cell receptor gene rearrangement were studied. Nodules consisted of atypical large cells that expressed CD30. Monoclonal rearrangement of T-cell receptors was observed in one case. Low-dose PUVA therapy may be associated with CD30+ large cell transformation in patients with MF.

Aged↗
PubMed1990

UV-B-induced bullous pemphigoid restricted to mycosis fungoides plaques.

We describe a 77-year-old female patient with plaque-stage mycosis fungoides (MF) who developed bullous lesions in lesional skin only, while receiving short-wave ultraviolet radiation (UV-B) therapy. Histopathological and immunohistochemical examination resulted in a diagnosis of bullous pemphigoid (BP). Withdrawal of the UV-B treatment and application of a high-potency topical corticosteroid cream resulted in a rapid regression of the BP. As the bullous lesions were strictly confined to the MF plaques, the cutaneous infiltrate was probably involved also in the development of the BP. To our knowledge, this is the second case report of the coexistence of MF and BP and the first one in which the BP might be UV-B-induced.

Aged↗
PubMed1995

Erythrodermic mycosis fungoides treated with total body irradiation and autologous bone marrow transplantation.

We describe a middle-aged man with aggressive erythrodermic mycosis fungoides whose responses to several well-established therapies for the disease were either poor or short-lived. Infusion of antibodies engineered against cells expressing the CD4 molecule produced little response. Total body irradiation coupled with autologous bone marrow transplantation led to an encouraging but unfortunately unsustained improvement.

Adult↗
PubMed1996

Poikilodermatous and verrucous mycosis fungoides.

Mycosis fungoides (MF) is a cutaneous lymphoma of presumed T-helper cell origin. It usually presents with patches and infiltrated plaques, but a wide range of atypical forms have also been described. We report a 48-year-old man who had a 2-year history of a pruritic, warty plaque on the right palm with surrounding poikilodermatous changes. He subsequently developed additional macules of poikiloderma atrophicans vasculare (PAV) on the right upper limb and abdomen. Biopsies from the arm showed poikilodermatous changes, and the warty plaque had features of MF. Verrucous or hyperkeratotic lesions appear to be an uncommon presenting feature of MF with few case reports in the literature to date. This presentation may be associated with considerable diagnostic delay, and MF should be considered in the differential diagnosis of acquired verrucous lesions. This case also illustrates that PAV may herald cutaneous malignancy, and that patients with this condition require close follow up.

Hand Dermatoses↗
PubMed2004

A randomized cross-over study to compare PUVA and extracorporeal photopheresis in the treatment of plaque stage (T2) mycosis fungoides.

PUVA is a well-established and effective treatment for plaque stage mycosis fungoides (MF) but its use is limited on a long-term basis because of the risk of cutaneous carcinogenesis. A further disadvantage is that nonexposed areas (sanctuary sites) often develop persistent disease. Therefore it is important to find alternative methods of treatment. Extracorporeal photopheresis (ECP) is a form of photochemotherapy that involves exposure of white blood cells to UVA with psoralens and can be effective in Sézary syndrome and erythrodermic cutaneous T-cell lymphoma. The aim of this study was to compare the efficacy of PUVA and ECP in the treatment of patients with T2 plaque stage (Stage 1B) MF who had a detectable peripheral blood T-cell clone. The study was of a cross-over design. Sixteen patients were randomized to receive either PUVA twice weekly for 3 months followed by ECP once monthly for 6 months at relapse, or vice-versa. Response was assessed by monthly skin scores and peripheral blood T-cell clonality. Ten patients received PUVA initially and six ECP initially. Eight patients completed the study. Skin scores taken at the completion of each treatment arm in patients who completed the study were 113 units better (confidence interval, 42-184 units) following 3 months PUVA than 6 months ECP (P = 0.002). Peripheral blood T-cell clones were detectable in all patients post-treatment. This study indicates that ECP is not effective in the treatment of plaque stage (1B/T2) MF even in patients with molecular evidence of a peripheral blood T-cell clone. Although PUVA was more effective than ECP, neither treatment modality cleared malignant T-cells from the peripheral blood.

Adult↗
PubMed2005

Narrow-band UVB in the treatment of early stage mycosis fungoides: report of 16 patients.

Although early stage mycosis fungoides (MF) has a generally good prognosis, and long-term survival rates with current therapies (UVB, photochemotherapy, topical nitrogen mustard, electron beam radiotherapy) are similar, there is concern regarding their potential side effects. It has been reported that the same effective UVB dose is safer than PUVA in terms of carcinogenicity, and that it produces fewer side effects. Our aim was to evaluate the effect of narrow-band UVB in the treatment of early stage MF. Sixteen patients (seven males, nine females; mean age, 40 years) with early stage MF received TL-01 phototherapy three times per week using a standard protocol. Twelve patients (75%) had complete response in a mean of 27.9 treatments, three had partial response, and one no response. Upon discontinuation of treatment, six patients with complete response relapsed in a mean time to relapse of 4.5 months. The present study indicates that narrow-band-UVB is an effective treatment modality for early stage MF.

Adult↗
PubMed2006

Long-term clearance of patch-stage mycosis fungoides with the 308-nm laser.

In order to investigate the effectiveness of the 308-nm xenon chloride laser in the treatment of patch-stage mycosis fungoides that is refractory to topical steroids, we conducted a prospective, histologically controlled, open-label pilot study. Eight patients with stage 1A or 1B cutaneous T-cell lymphoma that was unresponsive to application of potent topical steroids received 20 treatments with the laser over 10 weeks and were then followed up for a minimum of 30 months. Three patients had a complete clinical and histological response and remained free of disease after 30 months A further three patients had complete clinical and histological clearance of disease, but experienced relapse within the 30 months follow-up period. One patient withdrew from the study and one had a partial response. Further research to establish the role of the 308-nm laser in the treatment of MF appears justified.

Adolescent↗
PubMed1984

Woringer-Kolopp disease. A form of mycosis fungoides.

Woringer-Kolopp disease usually manifests as a solitary cutaneous patch or several patches confined to a single body region. While most cases have a good prognosis, there have been rare reports of cases with an aggressive course and a fatal outcome. The authors present two women who are alive with this condition, which, they believe is a variant of mycosis fungoides.

Adult↗
PubMed1991

Mycosis fungoides with oral involvement.

Oral mucous membrane involvement occurs uncommonly in mycosis fungoides and reports of it are scarce. We present two patients with severe involvement of the oral mucous membranes.

Aged↗
PubMed2005

Mycosis fungoides and Sezary syndrome: therapeutic approach and outcome in 113 patients.

BACKGROUND: Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common forms of cutaneous T-cell lymphoma (CTCL). Various topical and systemic therapeutic alternatives are available, but there is no standard or definite curative treatment regimen. When making a decision about the appropriate treatment modality, the age and compliance of the patient, stage of the disease, treatment accessibility, and previous treatment history should be considered. AIM: To determine the therapeutic response of patients with MF and SS to different treatment modalities. Patients were evaluated with respect to their clinical and demographic features. METHODS: One hundred and thirteen patients diagnosed clinically and dermatopathologically with MF and SS between March 1984 and June 2001 were included in the study. RESULTS: Of the 113 patients studied, 110 had a diagnosis of MF and three had a diagnosis of SS; 101 patients (89.4%) were diagnosed with early stage (IA, IB, IIA) and 12 (10.6%) with late stage (IIB, III, IVA, IVB) disease. The age at diagnosis varied between 12 and 81 years (mean, 45.6+/-15.8 years). Fifty-five (48.7%) patients were male and 58 (51.3%) were female. The duration of the skin lesions varied between 1.5 months and 32 years (mean, 6.1 years). Psoralen plus UVA (PUVA) was the most commonly used initial treatment modality in early stage disease (91%), with a complete remission (CR) rate of 80.4%. With PUVA+interferon-alpha (INF-alpha) treatment, CR was 57% in the early stages and 33.3% in the late stages. For late stage disease, systemic therapies, such as pentostatin, gemcitabine, and fludarabine, alone or in combination with INF-alpha, were preferred. Of the 113 patients, eight (7% of the total and 57.1% of the advanced stage cases) died of MF; 21.4% of the late stage patients showed partial remission and 14.2% showed CR. None of the patients diagnosed with early stage disease died of MF, but two (1.9%) progressed to late stage disease. CONCLUSIONS: PUVA and PUVA+INF-alpha are effective treatment modalities, especially for early stage MF. Once the disease has progressed, both MF and SS are very resistant to treatment regimens, including chemotherapeutic agents. It is important to diagnose and treat these diseases, especially MF, in the early stages for lasting remission.

Adolescent↗
PubMed2005

Mycosis fungoides associated with intestinal mucosa-associated lymphoid tissue lymphoma.

Coexistence of cutaneous T-cell lymphoma (CTCL) and a B-cell malignancy is rare. We report a case of mycosis fungoides (MF) associated with intestinal mucosa-associated lymphoid tissue (MALT) lymphoma, which is a low-grade B-cell lymphoma of marginal cell origin. To the best of our knowledge, there have been only two cases of coexistent CTCL and MALT lymphoma, and this is the first case with typical MF and intestinal MALT lymphoma. The coexistence in our case might be coincidental, but genetic predisposition and the immunoregulatory capacity of neoplastic T lymphocytes may be factors contributing to this association.

Antigens, CD20↗
PubMed2006

Microscopic study of normal skin in cases of mycosis fungoides.

BACKGROUND: During therapy of patients with mycosis fungoides (MF) at the Department of Dermatology, Kasr El-Aini Hospital, follow-up biopsies are routinely taken every 2 months. It was noticed that lesions of MF might become clinically normal during treatment, and yet still show microscopical evidence of MF. This finding raised the possibility that clinically normal skin in MF could be microscopically involved. AIM: The aim of our work was to evaluate the degree of histopathological involvement of normal-looking skin in patients with MF. PATIENTS AND METHODS: Thirty patients with stage IB were biopsied from their normal skin. Two biopsies were taken: one proximal (2 cm) and the other distal (> 5 cm) from any visible lesion. Ten normal controls were included in the study. All specimens were stained with H&E and examined microscopically. The microscopical diagnosis was confirmed by immunophenotyping. RESULTS: Epidermotropism was detected in 21 (70%) of the proximal skin biopsies and 14 (47%) of the distal skin biopsies, whereas no biopsy from the control group showed epidermotropism. All the proximal skin biopsies showed dermal infiltrate and 90% of the biopsies from the distal normal skin showed dermal infiltrate (mostly superficial perivascular). CONCLUSION: Normal skin in patients with MF could be affected microscopically and this may raise questions regarding the credibility of the current staging classification of MF, and may necessitate taking biopsies from normal skin before starting topical treatment. During MF treatment, biopsies from cured lesions are required before starting withdrawal.

Adult↗