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At least 1,135 records · Page 63Linked to original sources
PubMed1999

Low dose interferon-alpha2b combined with PUVA is an effective treatment of early stage mycosis fungoides: results of a multicenter study. Cutaneous-T Cell Lymphoma Multicenter Study Group.

BACKGROUND AND OBJECTIVE: The early stages of mycosis fungoides (MF) can be treated but not cured by photochemotherapy (PUVA) alone; some recent studies of the effect of a combination of human interferon-alpha (IFN(alpha)) and PUVA reported a high degree of response. The aim of our study was to evaluate the activity of a low dose of IFN-alpha2b combined with PUVA. DESIGN AND METHODS: Twenty-five patients were included: 16 men and 9 women aged between 23-80 years; 19 patients ahd stage I and 6 stage II disease. In the induction phase, the dose of IFNalpha was gradually raised over 6-8 weeks to the target dose of 18 MU/week; in the maintenance phase, the combination with PUVA allowed IFNalpha to be reduced to a maximum dose of 6 MU/week; in this way the cumulative administration of IFNalpha and PUVA was considerably lower than in similar combination protocols. Treatment success was analyzed in terms of freedom from treatment failure (FFTF). RESULTS: After the induction phase 9/25 patients (36%) achieved complete remission (CR) and 15/25 (56%) achieved partial remission (PR). One to five months from the beginning of the maintenance phase, a CR was recorded in 19/25 patients (76%) and a PR in 5/25 patients (20%) accounting for an overall response rate of 96%. The median of FFTF was not reached; probability of FFTF was 82% at 12 months and 62% at 24 months. Disease free survival projected to 48 months was 75%. INTERPRETATION AND CONCLUSIONS: Even with low doses of IFNalpha plus PUVA it is possible to achieve excellent clinical responses,many of which are long-lasting, in patients with early MF.

Adult↗
PubMed2000

Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases. French Study Group of Cutaneious Lymphomas.

The course of mycosis fungoides (MF) is indolent except when transformation to a large T-cell lymphoma occurs. The diagnosis of transformed MF (T-MF) relies on the presence of more than 25% of large cells on biopsy of an MF lesion. We analyzed 45 patients with T-MF recorded by the French Study Group on Cutaneous Lymphomas to better determine clinicopathological features of MF transformation and to analyze their impact on prognosis. Median time from diagnosis of MF to transformation was 6.5 years. Extracutaneous progression was present in 20 patients. Mean survival from transformation to death was 22 months. In univariate analysis, only an extracutaneous progression was associated with a worse prognosis (5-year actuarial survival: 7.8% versus 32%). Neither sex, age, clinical and skin disease stage at transformation, transformation speed, nor percentage of large cells or CD30 expression (14 of 45) had a prognostic value. When performing multivariate analysis, age (at least 60 years), and extracutaneous spreading were found to be associated with a poor prognosis. There was no difference between survival curves of patients with T-MF and with pleomorphic large T-cell CD30- lymphomas. The main diagnostic pitfall was "histiocytic-rich" MF, requiring CD68 staining for the diagnosis of T-MF. Out of 45 patients, 6 presented an histologic transformation before clinical progression, suggesting that an early histopathological diagnosis may be performed by histological follow-up. The prognostic value of such early histopathological diagnosis must be confirmed by prospective studies.

Adult↗
PubMed2000

[Clinicopathological differential diagnosis of mycosis fungoides/Sézary syndrome from the cutaneous type of adult T-cell leukemia/lymphoma].

Mycosis fungoides/Sézary syndrome(MF/SS) is considered as a distinct clinical entity in the latest classifications, including the revised European-American classification of lymphoid neoplasm(REAL), and the ones by the WHO and the European Organization for Research and Treatment of Cancer(EORTC). In Japan, where is an endemic area for human T-cell lymphotropic virus type 1(HTLV-1), we have been facing some problems concerning the differential diagnosis of MF/SS. We have observed many cutaneous types of adult T-cell leukemia/lymphoma(cATL/L) that can initiate as erythematous infiltrating plaques similar to MF or as an erythrodermic lesion similar to SS and also as tumoral and nodular lesions, solitary or multiple that resemble d'emblée type MF. The importance of the differential diagnosis between MF/SS and cATL/L lies on the significant difference on the prognosis. It is worth to differentiate MF/SS from cATL/L, especially in MF patients positive for HTLV-1 antibody. We emphasize on the characteristics that differentiate MF/SS from cATL/L as well as on the new findings regarding clinical, histopathological, phenotypical and genotypical aspects of MF/SS.

Antibodies, Viral↗
PubMed1979

Mycosis fungoides: electron beam therapy in England.

Since 1962, total-skin electron-beam therapy has been available in London, England, for the treatment of patients with mycosis fungoides and skin lymphomas. The 6-mev linear accelerator produces a pencil beam of electrons at 7 mev, which are scattered through a brass scatterer and decelerated by either of two carbon decelerators or a copper decelerator to produce a beam of effective energy of 2.5, 3, or 3.5 mev; these beams have an 80% isodose distribution at 5.5, 8, and 11.5 mm, respectively. The patient receives 200 rads to the anterior, posterior, and right and left lateral fields, for a total dose of between 1600 and 2600 ras in ten treatments over 5--7 weeks. Complete clearing of the disease can be predicted in all patients except those with the most advanced tumors. However, the duration of remission after electron-beam therapy is approximately 18 months and we are presently investigating the combination of psoralens and ultraviolet light therapy as maintenance treatment following lower-dose electron-beam therapy.

Humans↗
PubMed1979

Combined therapy for patients with mycosis fungoides.

In a comprehensive mycosis fungoides program, 60 patients have been seen with a pathologic diagnosis of this disease. Forty-four patients with advanced disease were referred for radiation therapy. Three treatment techniques were identified in which 14 patients were treated with localized fields using electrons or whole-body electron-beam therapy with doses of less than 3000 rads, 21 patients were treated using the Stanford technique with tissue doses of between 3000 and 4000 rads, and nine patients were treated with six cycles of mechlorethamine, vincristine, prednisone, and procarbazine or cyclophosphamide, vincristine, prednisone, and procarbazine following the electron-beam therapy. The actuarial survival rate was 45% at 1 year for the 14 patients with localized electron-beam therapy, whereas the actuarial survival rates were 83% for patients treated with whole-body electron-beam therapy and 100% for patients treated with whole-body electron-beam therapy followed by four-drug chemotherapy. The recurrence-free interval for these three groups correlates with these observations. A central nervous system recurrence has been observed in the combined-therapy group.

Adult↗
PubMed1979

A 10-year experience with topical mechlorethamine for mycosis fungoides: comparison with patients treated by total-skin electron-beam radiation therapy.

A group of 243 patients with mycosis fungoides (MF) received treatment with topical applications of dilute aqueous solutions of mechlorethamine and/or systemic chemotherapy over the past 10 years. The likelihood of a complete and relapse-free remission and survival was found to correlate inversely to the magnitude of disease as denoted by a simple staging system. Although disease-free intervals of greater than 3 years have occurred thus far in 32 (13%) patients, the permanency of these remissions and the curability of disease remain uncertain because of the variability of disease progression characteristic of MF. Comparison of treatment results with those published on a large group of patients treated with total-skin electron-beam radiation therapy indicates that the chemotherapeutic approach to the treatment of MF is equally effective in promoting survival.

Administration, Topical↗
PubMed2000

[Mycosis fungoides terminating in acute myelocytic leukemia].

A 57-year-old woman was admitted to our hospital with suspected leukemia in September 1999. In 1990, systemic erythema had occurred, and mycosis fungoides (MF) had been diagnosed by skin biopsy. Interferon-gamma therapy had not been effective, and the erythema had disappeared after treatment with psoralen and ultraviolet A (PUVA) therapy (1.46 J/cm2). The patient had subsequently done well with a course of topical steroids. On admission this time, the WBC count was 1,600/microliter with 6% blasts. The total nucleated cell count in a bone marrow aspirate was 43.1 x 10(4)/microliter, of which 86.2% were peroxidase-positive blasts. Acute myelocytic leukemia (AML) was diagnosed. Chromosomal analysis demonstrated abnormalities of 48, XX, +4, +8, +add(10)(p11), add(11)(q23) in 10 of 20 cells, and 51, idem, +6, +8, +21, +mar in 8 cells with mixed-lineage leukemia gene rearrangement. Therapies (radiation, chemotherapy and PUVA) for MF, and the altered immune response seen in patients with this disease, especially in the more advanced stages, collectively termed cutaneous T-cell lymphoma (CTCL), suggest that such patients may be at increased risk of a second primary malignancy. To our knowledge, AML has been reported in 8 MF patients including the present one. Attention should be given to the possibility of MF terminating in AML.

Female↗
PubMedDate not supplied

Evaluation of T-cell receptor gene rearrangements in patients with recurrent patch/plaque (T2) CTCL (mycosis fungoides).

Cutaneous T-cell lymphoma is typically a clonal neoplasm of epidermotropic CD4+ T-lymphocytes that includes the entity mycosis fungoides (MF). After identification of patients with recurrent MF treated with total skin electron beam therapy (TSEBT) at the Yale University School of Medicine, this study attempted to compare T-cell receptor (TCR) gamma gene rearrangements via polymerase chain reaction (PCR) in both original and recurrent skin biopsies from these patients. Between 1974 and 1996, a total of 95 T2 MF patients were treated with TSEB, and four of these were identified for the study. Slides and tissue samples of both primary and recurrent skin biopsies for each patient were confirmed as being consistent with ME DNA for PCR was isolated from paraffin-embedded tissue samples. Using consensus primers that hybridize with conserved regions of the TCR gene, these regions of the genome were amplified. The PCR products were then analyzed by acrylamide gel electrophoresis. Of the primary and recurrent samples from four patients with a median disease-free interval (DFI) of 1222 days, only two showed evidence of a dominant TCR clone. A number of factors, including lack of sequence homology between the primers and the gene segments, the existence of multiple neoplastic cell lines, DNA degradation in the archival samples, and the presence of reactive as well as malignant lymphocytes, may have prevented the detection of dominant TCR rearranged clones in the samples. Despite the results of this study, TCR analysis via PCR and gel electrophoresis continues to be of utility in the evaluation of patients with MF when used in conjunction with other diagnostic modalities and in cases with nonspecific clinical, histopathological, and immunophenotyping findings.

Adult↗
PubMed2001

Carbamazepine hypersensitivity syndrome mimicking mycosis fungoides.

We report a case of a patient who experienced a generalized skin rash with systemic involvement 2 months after beginning carbamazepine treatment for trigeminal neuralgia. Skin biopsy specimens suggest mycosis fungoides. Complete remission of the clinical and pathologic changes after drug discontinuation and the positive result of carbamazepine from an epicutaneous test suggest a diagnosis of pseudolymphoma due to carbamazepine.

Aged↗
PubMed1975

Mechlorethamine desensitization in therapy for mycosis fungoides. Topical desensitization to mechlorethamine (nitrogen mustard) contact hypersensitivity.

Five patients with mycosis fungoides who had developed contact dermatitis to a nitrogen mustard, mechlorethamine hydrochloride, even in low concentrations (1 to 5 mg/100 ml), received daily total-body applications of extremely dilute solutions (0.01 to 0.1 mg/100 ml) of mechlorethamine. The concentrations of the drug were approximately doubled weekly if the patient could tolerate it, or they were raised more slowly if the patient could not. Attempts to desensitize one patient were discontinued since he was unable to tolerate a greater concentration than 1.0 mg/100 ml after trying for one year. Another patient was able to tolerate a concentration of 3 mg/100 ml after three months, at which time his skin had completely cleared and treatment was stopped. Three other patients were desensitized during a period of 8 to 13 months to the point of tolerating the full therapeutic concentration used in our clinic (20 mg/100 ml) without experiencing dermatitis or pruritus.

Administration, Topical↗
PubMed2001

Hypopigmented mycosis fungoides: a report of 7 cases and review of the literature.

Over the last 2 decades, hypopigmented macules have been reported with increasing frequency as an initial presentation of mycosis fungoides (MF). We retrospectively reviewed 7 patients with hypopigmented MF. The mean age was 35 years at disease onset, with a mean of 5.5 years' duration of illness before presentation. All of our patients were Fitzpatrick skin type IV or V, and most reported pruritus. Histologic findings in all cases were consistent with MF. Treatment with topical nitrogen mustard produced repigmentation in 4 of 6 patients.

Adult↗
PubMed2001

Fas ligand expression by neoplastic T lymphocytes mediates elimination of CD8+ cytotoxic T lymphocytes in mycosis fungoides: a potential mechanism of tumor immune escape?

Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma (CTCL) and arises from the accumulation and clonal proliferation of epidermotropic, CD4+/CD45RO+ (helper/memory) T lymphocytes. Loss of CD8+ CTLs within MF lesions is associated with poor prognosis and disease progression. Because T-lymphocyte apoptosis is controlled mainly through the Fas/Fas ligand (FasL) pathway and tumor cells may escape immune surveillance by expressing FasL, triggering apoptosis of tumor-infiltrating T lymphocytes, we studied the role of this system in MF. T-cell subsets, Fas/FasL expression, and apoptosis were evaluated in normal and lesional skin biopsy specimens from 21 patients with all stages of MF and in cultured CTCL cell lines (MJ, HUT78, and HH) using immunohistochemistry, terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL), and Western blotting. MF lesions and paired, clinically "normal," uninvolved skin showed increased numbers of both TUNEL-positive epidermal keratinocytes (n = 13; F = 31.146; P < 0.01, ANOVA) and dermal lymphocyte infiltrates (n = 13; F = 15.825, P < 0.01, ANOVA) compared with the normal control skin. FasL expression was highest in lesional epidermal keratinocytes, in CTCL tumor cell lines, and in dermal tumor lymphocytes in MF lesions compared with uninvolved skin. FasL colocalized with CD45RO+ cells. CD8+ cells in or adjacent to CD45RO+ cells were positively labeled by TUNEL for apoptosis. In addition, CD8+ cell numbers were decreased in areas in which FasL+ tumor cells were abundant (2.01 +/- 0.86%) compared with non-FasL expressing areas (13.53 +/- 3.54%; P < 0.02). These results suggest that a potential mechanism of tumor immune escape in MF is FasL-mediated apoptosis of infiltrating CD8+ CTLs.

Adult↗
PubMed2001

[Interferon-alpha and PUVA therapy for mycosis fungoides].

14 patients suffering from early stage mycosis fungoides were treated with interferon alpha 2-a and PUVA/1 patient in stage I a, 3 patients in stage I b, 4 patients in stage II a and 6 patients in stage II b/during 3-21 months time course. Interferon alpha 2-a was administered 3 times a week, in escalating dose from 3 MU to 9 MU, determining the individual maximal tolerated dose. All of the patients responded well to the treatment. Partial remission was observed after 4-13 weeks of treatment. Total remission developed in 8 cases, after 8 weeks- 9 months of the treatment. Side effects occurred frequently: weight loss, pain, fever, fatigue, leucopenia, thrombopenia, liver enzyme elevation. Because of the side effects the dose of the interferon was reduced individually, the dose reduction did not cause relapse.

Aged↗
PubMedDate not supplied

Peripheral blood involvement in a mycosis fungoides patient with limited skin lesions: phenotypical features and homing molecule pattern.

Peripheral blood involvement in mycosis fungoides (MF) patients is more frequent in the advanced stages and is associated with a worse prognosis. We report a MF patient with limited patch lesions on her shoulders, upper chest and thighs (T2N0M0) and peripheral blood involvement. Clonality in the peripheral blood was demonstrated by the PCR assay and confirmed by the expansion of the same restricted variable region of the TCR beta-chain (vbeta17) expressed in the cutaneous infiltrate. The patient was treated with fludarabine achieving a complete hematological response followed by an early relapse, whilst the cutaneous lesions remained unchanged. The soluble interleukin-2 receptor levels showed a decrease from baseline levels down to normal values at hematological remission, followed by a further increase. The low sLex/CLA expression in the cutaneous lymphoid infiltrate could have given rise to a higher recruitment in the peripheral blood.

Aged↗
PubMedDate not supplied

Nevoid hyperkeratosis of the areola with histopathological features mimicking mycosis fungoides.

Hyperkeratosis of the areola is a rare benign condition of unknown etiology characterized by slowly growing verrucous thickening and brown pigmentation of the areola or/and the nipple. It may be presented as isolated nevoid form or associated with other skin diseases. We described a 21-year-old woman with characteristic lesions of nevoid hyperkeratosis in both areolas but with histopathological findings resembling mycosis fungoides. Cutaneous lesions have remained unchanged after two years of follow-up.

Administration, Topical↗
PubMed2002

Remission of a cutaneous Mycosis fungoides after topical 5-ALA sensitisation and photodynamic therapy in a patient with advanced HIV-infection.

BACKGROUND: Treatment of Mycosis fungoides (MF) in HIV-infected patients is controversially discoursed. Photodynamic therapy (PDT) after topical sensitization with 5-aminolevulinic acid (5-ALA) is a new and effective modality for treatment of skin malignancies. OBJECTIVE: In this report we describe, what is, to our knowledge, the first case of a patient with MF through advanced HIV-infection, successfully experiencing topical 5-ALA sensitization and PDT. METHODS: 5-ALA ointment was applied to plaques and held in occlusion for 4 hours. PDT was applied using the PDT 1200 irradiation source (Waldmann Medizintechnik System) with 180 J/cm superset 2. RESULTS: Complete remission of MF was achieved, after two completed cycles of photodynamic therapy. CONCLUSION: MF lesions in the presended case showed a high response to 5-ALA sensitization and PDT. This modality appeared to be very effective in treatment of MF in a HIV-infected patient and could be a valuable treatment option for cutaneous T-cell lymphoma in HIV-infected patients.

Acquired Immunodeficiency Syndrome↗
PubMed1992

Cutaneous expression of Thy-1 in mycosis fungoides.

Dermal dendritic cells from eleven cases of mycosis fungoides (MF) (six patch and five plaque stage), two cases of pre-MF, and five specimens of normal human skin, were characterized immunohistochemically using a panel of antibodies including anti-human Thy-1, intercellular adhesion molecule-1 (ICAM-1; CD54), endothelial leukocyte adhesion molecule-1 (ELAM-1), vascular cell adhesion molecule-1 (VCAM-1), CD1a, CD2, CD14, CD18, CD34, MAC387, KP-1, EBM-11, factor XIIIa, factor XIIIs, and S100. Thy-1 expression in normal skin was limited to the microvascular endothelium and perivascular dendritic cells. An extensive interstitial network of Thy-1+ dendritic cells was seen in the papillary dermis of all cases of MF, whereas no epidermal cells were Thy-1+. The mean +/- standard deviation of interstitial Thy-1+ cells per high power field in the dermis was: normal skin, 2.86 +/- 0.34; pre-MF, 15; patch stage MF, 13.4 +/- 7.08; plaque stage MF, 49.96 +/- 21.29. Thy-1+ dendritic cells morphologically resembled the factor XIIIa+ "dermal dendrocyte" (DD) and shared their VCAM-1+, ICAM-1+, CD1a, CD2-, CD14+, CD18+, EMB11+, factor XIIIa+, factor XI-IIs-, S100-, MAC387- and KP-1-immunophenotype in MF. Double labeling studies revealed up to 50% of Thy-1+DD were also factor XIIIa+ in MF. Immediately beneath these cells was a similar network of CD34+, Thy-1-, factor XIIIa- dendritic cells limited to the reticular dermis. Strong microvascular endothelial cell expression of Thy-1 and VCAM-1, and focal vascular ELAM-1 expression were also seen in MF. Distinct cellular compartmentalization (papillary dermis versus reticular dermis versus epidermis) of dendritic cells is demonstrated by the differential expression of Thy-1, factor XIIIa, and CD34 antigens. The extensive number and prominent dermal dendritic network in the papillary dermis juxtaposed between epidermal keratinocytes (KC) and dermal/epidermal T cells, suggests an important pathophysiologic role for this newly recognized and immunophenotypically distinctive cell population in MF.

Antigens, CD↗