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Results for “MYCOSIS FUNGOIDES”

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PubMed1991

Soluble IL2 receptor serum levels and epidermal cytokines in mycosis fungoides and related disorders.

We examined the immune activation in 20 patients with mycosis fungoides, 6 patients with erythrodermia of unknown origin (Pré-Sézary's syndrome), 5 with lymphomatoid papulosis, 4 with parapsoriasis, 2 with Sézary's syndrome, and 2 with actinic reticuloid, by measuring soluble interleukin-2 receptor levels in serum. In Mycosis fungoides we observed normal levels in 3 patients (less than 500 units/ml), between 500 and 1000 units/ml in 9 patients, and greater than 1000 units/ml in 5 patients. Four of these 5 patients died within one year after this observation, as did 2 patients with Pré-Sézary and Sézary's syndrome, respectively, who had a similarly large increase in sIL2R. Although sIL2R is not a specific parameter for cutaneous T-cell lymphoma, a value above 1000 units/ml is correlated with clinical disease activity and is a serious prognostic parameter. We also studied cytokine activity in epidermal homogenates from 9 patients with Mycosis fungoides and one patient with Sézary's syndrome. We observed interleukin-1-like activity within the normal range for healthy skin. However, we also observed in the same epidermal homogenates a T-lymphocyte chemotactic activity in patients with stage II, but not in stage I. The nature of this activity is not yet fully elucidated, but it may be an important biological factor for the epidermal T-cell accumulation in this disorder.

Aged↗

PubMed1979

Defective monocyte chemotaxis in mycosis fungoides: lack of essential helper lymphocytes.

The effect of lymphocytes on monocytes chemotaxis in vitro was studied using lymphocyte fractions prepared by glass bead column separation and mononuclear cell fractions prepared by Ficoll-Hypaque separation. The diminished monocyte chemotaxis in ten patients with mycosis fungoides was corrected in vitro by the addition of normal lymphocytes. This helper effect was not mediated by soluble factors. Lymphocytes from mycosis fungoides patients did not inhibit chemotaxis by monocytes from mycosis fungoides patients did not inhibit chemotaxis by monocytes from normal donors. No cell directed chemotactic inhibitor, differences in LDCF production in vitro or differences in circulating chemattractants were found. These data support the conclusion that the abnormal monocyte chemotaxis seen in mycosis fungoides is due to lack of essential helper lymphocyte function and represents an abnormal mononuclear cell interaction which may be important in the establishment of the mononuclear cell infiltrate in mycosis fungoides.

Adult↗

PubMed1977

Multiple osteolytic lesions in a patient with mycosis fungoides.

Skeletal lesions that were clinically significant and roentgenographically demonstrable developed in a patient with mycosis fungoides. Biopsy specimens from skin plaques and tumors and from a tibial tumor mass revealed an infiltrate of similar-appearing cells that were compatible with mycosis cells. Bone marrow involvement is not unusual in patients with mycosis fungoides with extracutaneous disease, but destruction of cortical bone in mycosis fungoides, as demonstrated by the patient in this report, is rare.

Biopsy↗

PubMed1995

[Mycosis fungoides bullosa].

We report on a male patient with mycosis fungoides (MF) with blisters mainly occurring on clinically uninvolved skin. This rare association has to be differentiated from other bullous diseases of the skin. The clinical picture has been known since the end of the last century and has been described as mycosis fungoides bullosa. The pathomechanism, however, has not yet been exactly identified. The different clinical and histological features of the disease are discussed.

Aged↗

PubMed1984

Malignant melanoma associated with mycosis fungoides.

2 cases of malignant melanoma in 2 male patients, 68 and 63 years old, associated with mycosis fungoides and parapsoriasis en plaques, respectively, are reported. The parapsoriasis later on developed into mycosis fungoides. A pathogenetic linkage based on decreased cellular immunity in mycosis fungoides is suggested.

Aged↗

PubMed1987

The indications for total cutaneous electron beam radiation therapy of mycosis fungoides.

From 1977 to 1984, we treated 34 patients with mycosis fungoides and 9 patients with B cutaneous lymphomas. Eighteen patients with mycosis fungoides were treated with total skin electron irradiation (TSEI) and had a minimum follow-up of 15 months (range 15 months to 7 years). The lowest electron energy of the linear accelerator was 8 MeV therefore we placed a plexiglas screen between the patient and the machine; the resulting electron energy was 4 MeV. The total dose was 30 Gy delivered in 12 fractions over 40 days. There were 8 males and 10 females. The median age was 48 years (ranging from 13 to 78 years). All patients were staged as follows: Stage A = superficial lesions covering less than 50% of the body surface; Stage B = superficial lesions covering more than 50% of the body surface; Stage C = tumors involving the skin, lymph nodes and/or visceral organs. Five patients with Stage A (5/5) and 5 patients with Stage B (5/5) had a complete remission, 1 stage A patient relapsed 6 months after completion of treatment. All the Stage B patients recurred between 3 and 15 months. The recurrences were localized to the skin and were well controlled with topical nitrogen mustard or puvatherapy. Among the Stage C patients, 3 did not respond to treatment and died of their disease; the remaining 5 patients achieved complete remission but they all relapsed from 2 to 9 months following completion of treatment. The median follow-up was 32 months and the average time for relapse was 6.5 months. All relapses except one (15 months) occurred within the first year. We feel that total skin electron irradiation is indicated in Stage A and B patients. However, we feel Stage C patients should receive TSEI for palliative purposes only.

Adolescent↗

PubMed1981

E-rosette inhibitory factor in sera from patients with mycosis fungoides.

Peripheral blood lymphocytes from some of patients with mycosis fungoides disease showed decreased ability to form rosettes with sheep erythrocytes. This decreased percentage of E-rosette forming cells could be normalized when those cells were incubated in culture for 20 hr. Since these data led us to considering a possible inhibitory factor present in patients' sera, we tested their ability to inhibit E-rosetting by T lymphocytes from normal donors, and found that sera from mycosis fungoides patients with low levels of E-rosetting blood lymphocytes showed greater inhibitory effect on E-rosette formation by normal T cells when compared to those either from normal donors or from mycosis patients who had almost normal levels of E-rosetting blood lymphocyte number. The E-rosette inhibitory factor was sensitive to 2-mercaptoethanol treatment and was copurified with serum IgM by ammonium sulfate precipitation and by sequential gel filtrations, suggesting that it might be an anti-T lymphocyte antibody naturally occurring during the disease process.

Adult↗

PubMed2001

[Transformation of mycosis fungoides to pleomorphic T-cell lymphoma and central nervous system involvement].

INTRODUCTION: Although mycosis fungoides is a malignant T-cell lymphoma involving mainly the skin, neurological complications are possible, with a poor prognosis. EXEGESIS: A 59-year-old man, treated for mycosis fungoides with transformation to a pleomorphic T-cell lymphoma for 1 year, was seen for mental status changes with confusion. A brain parenchyma localisation was found. CONCLUSION: This observation emphasizes the exceptional neurological tropism in the patients with mycosis fungoides. A transformation to a more aggressive cutaneous T-cell lymphoma seems necessary to induce a central nervous system involvement.

Brain Neoplasms↗

PubMed1981

Focal contact sensitivity to nitrogen mustard in lesions of cutaneous T-cell lymphoma (mycosis fungoides).

A report is presented on a patient with mycosis fungoides who developed a remarkable focal contact sensitivity to topically applied nitrogen mustard in aqueous solution (1/5000). Although the nitrogen mustard was applied over the entire skin surface, repeated challenge demonstrated that the eczematous contact dermatitis reaction was limited precisely to the areas of clinically identifiable mycosis fungoides. The surrounding normal skin showed no response. The basis for the surprising focal induction and localization of contact dermatitis is not known but might reflect the presence of a clone of malignant T-lymphocytes which became specifically sensitized to the nitrogen mustard.

Administration, Topical↗

PubMed1985

Bullous pemphigoid. Occurrence in a patient with mycosis fungoides receiving PUVA and topical nitrogen mustard therapy.

A 57-year-old woman with mycosis fungoides developed blisters within cutaneous plaques while receiving PUVA therapy and topical nitrogen mustard. Direct and indirect immunofluorescence studies showed the findings of bullous pemphigoid. Her bullous disease was controlled after cessation of these therapies and institution of prednisone and methotrexate. During the 5 months following completion of a course of electron-beam therapy, she has been free of the cutaneous manifestations of both diseases. Previous instances of PUVA-related pemphigoid have occurred in psoriatics. The role of ultraviolet light in the induction of pemphigoid is discussed, particularly with regard to its possible interaction with the altered skin of psoriasis or mycosis fungoides. Some of the rare cases of bullous mycosis fungoides might actually have represented ultraviolet-unmasked bullous pemphigoid.

Administration, Topical↗

PubMed1981

Dermatopathic lymphadenopathy. Comparison of cases associated and unassociated with mycosis fungoides.

Thirty-three biopsies showing dermatopathic lymphadenopathy were obtained from patients with documented cutaneous mycosis fungoides and were studied together with an equal number of dermatopathic lymph nodes derived from patients without evidence of mycosis fungoides. The nodes were evaluated for a variety of histologic features including mitotic figures, immunoblasts, and in particular for the number of atypical cerebriform lymphocytes. Atypical lymphocytes were found to be equally as frequent among both groups of dermatopathic lymph nodes without any statistically significant differences in quantitation or distribution. Similarly, no other morphologic variable was found which would allow an objective distinction of dermatopathic lymphadenopathy from patients with or without mycosis fungoides.

Adolescent↗

PubMed1988

Light-microscopic assessment of 100 patients with patch/plaque-stage mycosis fungoides.

The light-microscopic recognition of patch/plaque stages of mycosis fungoides is difficult, but remains as the "gold standard" for the diagnosis of mycosis fungoides (MF). A review of previous publications concerning the light-microscopic histological criteria for recognition of MF is followed by a summary of our recent histological findings involving 100 patients. The quantitative histological assessment included 13 different parameters for each case of patch/plaque-stage disease. The different criteria included parakeratosis, acanthosis, density of upper dermal mononuclear cell infiltrate, degree of lymphocyte atypia, degree of epidermotropism, Pautrier microabscess formation, band-like pattern, presence of basal vacuolar change, spongiosis, papillary dermal fibrosis, presence of eosinophils, and presence of plasma cells. The most significant histological features of patch/plaque-stage MF are: 1. Broad zones of epidermis in which lymphocytes, which are usually not markedly atypical, are in close apposition to basal and lower-level keratinocytes predominantly as single cells in a somewhat linear configuration on the epidermal side of the dermal-epidermal junction. In general, the surrounding keratinocytes do not display any cytopathic changes, i.e., basal vacuolar degeneration or apoptosis. 2. The papillary dermis contains a variably dense mono-nuclear cell infiltrate that is usually polymorphous and in which there is papillary dermal fibrosis with plasma cells and eosinophils. 3. Pautrier microabscesses are only present in the minority of cases of MF. Representative "threshold" cases of the earliest recognizable diagnostic cases of MF are illustrated. A pragmatic diagnostic approach to patients presenting with a clinical picture suggesting MF is outlined. The potential usefulness of additional ancillary diagnostic techniques such as immunoperoxidase frozen-section stains and T-cell-receptor gene rearrangements are briefly reviewed.

Adult↗

PubMed1982

Transfer factor therapy in mycosis fungoides: a double-blind study.

Sixteen patients with mycosis fungoides (MF) were given either active transfer factor (TF) or heat-inactivated TF as additional therapy to topical nitrogen mustard or PUVA. The TF was prepared from non-selected healthy blood donors. The clinical evaluation after 2 years of therapy showed that among 8 patients treated with active TF, none went into complete remission of their disease 4 patients had partial remission, one was unchanged, 2 progressed, and one died of active MF. In the placebo-treated group, 5 patients achieved complete remission and 2 partial remission. One patient died early in the trial due to cardiac disease. Immunological studies during the first year of therapy revealed cutaneous anergy towards tuberculin in most patients. This anergy did not change during TF therapy and differed from normal lymphocyte reactivity in vitro after tuberculin stimulation. At the start of treatment the patients had diminished levels of T lymphocytes in peripheral blood. A temporary increase was observed in the total number of T lymphocytes in patients after one month of treatment with active TF. After one year the T lymphopenia had disappeared in both groups. The mitogen reactivity of lymphocytes was found to be normal (PHA, PWM) or somewhat reduced (Con A). It is concluded that under the conditions employed in this trial, TF was not able to prevent progression of early mycosis fungoides, when viewed over a period of 2 years.

Administration, Topical↗

PubMed1995

CD8-positive tumor-infiltrating lymphocytes influence the long-term survival of patients with mycosis fungoides.

BACKGROUND: Nonneoplastic mononuclear cells commonly infiltrate lesions of mycosis fungoides. OBJECTIVE: We sought to determine the immunophenotypic characteristics of these cells and to determine whether the presence of CD8+ tumor-infiltrating lymphocytes has an impact on prognosis. METHODS: Skin biopsy specimens from 78 patients were stained with immunopleroxidase techniques to determine their phenotypic characteristics. The proportion of CD8+ tumor-infiltrating lymphocytes was quantified and compared with stage of disease and survival rate. RESULTS: Patients with more limited T-stage disease tended to have a higher proportion of CD8+ cells in their skin biopsy specimens, compared with patients with more advanced T-stage disease. Within each T-stage patients with a larger proportion of CD8+ cells had a better survival rate than those with fewer CD8+ cells (p < 0.05 for T1 and T3). A multivariate analysis confirmed the importance of T stage (p = 0.0006), overall stage (p = 0.0112), and CD8 positivity (p = 0.0335) in this cohort of patients. CONCLUSION: CD8+ tumor-infiltrating lymphocytes in mycosis fungoides correlate with improved survival rate and may exert an antitumor effect rather than being mere bystander cells.

Actuarial Analysis↗

PubMed1992

Total skin electron beam radiation therapy for mycosis fungoides.

Forty-nine patients with biopsy-proven mycosis fungoides, Stages I-IV were treated using total skin electron beam irradiation (TSEBI). Total dose ranged from 600 cGy to 3,200 cGy. To evaluate the dose response relationship, patients were retrospectively divided into two groups. In Group I, 18 patients received a dose of 2,000 cGy or less, and in Group II, 31 patients received more than 2,000 cGy. The overall response rate was 87.7% with a 75.7% complete response and 12.2% partial response. Complete response was higher among patients with early stage disease: (Stage IA 1/1, Stage IB 23/35 (92%), Stage IIA 3/4 (75%), Stage IIB 4/8 (50%), Stage III 3/6 (50%), Stage IVA 1/1, Stage IVB 0/1, and unstaged group 2/3 (66.6%)). Patients treated with a higher total dose had a higher overall 5-year survival rate (Group I 38%, Group II 68%), longer median duration of complete response (Group I, 27 months; Group II, 35.3 months), slightly better complete response rate (72.2% for Group I, 77.4% for Group II), and lower recurrence rate (Group I, 94%; Group II, 83.9%) compared to patients with lower total dose. Complications from TSEBI were minimal. Total skin electron beam irradiation is effective in controlling early stage mycosis fungoides; however, a prospective study to evaluate optimum total dose is needed.

Adult↗

PubMed1992

Mycosis fungoides mimicking perioral dermatitis.

A 71-year-old woman under PUVA-treatment for mycosis fungoides developed erythematous patches around the nasolabial folds and papules on the chin with clinical features of perioral dermatitis. Histology showed a specific infiltrate of mycosis fungoides with predominance of medium to large-sized pleomorphic lymphocytes and immunoblasts. Immunohistochemical analysis revealed the T-phenotype of the neoplastic cells. Small clusters of B-lymphocytes could also be observed within the infiltrate. Perioral dermatitis-like lesions can be added to the spectrum of rare and unusual clinical manifestations of mycosis fungoides.

Aged↗

PubMed1989

Mycosis fungoides in relation to environmental exposures and immune response: a case-control study.

Mycosis fungoides is a cutaneous T-cell lymphoma of unknown etiology, thought to be a rare sequela of chronic antigenic stimulation that may occur, for example, with exposure to contact allergens. To explore this possibility, we interviewed 174 patients with mycosis fungoides and 294 randomly selected control subjects in the San Francisco, Los Angeles, and Seattle areas concerning their lifetime histories of employment, chemical exposures, allergy, atopy, and certain medical conditions. Patients reported higher prevalence of cancers other than the non-Hodgkin's lymphomas and skin cancers (relative risk = 3.3, P less than .001) and were more likely than controls to burn when exposed to the sun (for nonblacks, relative risk = 1.7, P = .01). The latter difference may reflect a manifestation rather than a precursor of the disease. We found no consistent or biologically plausible differences between patients and controls with respect to types of jobs held, or to occupational or vocational exposures to chemicals. These findings do not support the hypothesis that persistent antigenic stimulation by contact allergens is etiologically important in the pathogenesis of mycosis fungoides.

Case-Control Studies↗

PubMed1987

Combination chemotherapy with bleomycin, cyclophosphamide, prednisone and etretinate (BCPE) in advanced mycosis fungoides: a six-year experience.

A six-year experience in 20 patients with advanced mycosis fungoides treated with combination chemotherapy with bleomycin, cyclophosphamide, prednisone and etretinate (BCPE) in advanced mycosis fungoides showed initial complete remissions in 16 patients (85%). The initial complete remissions lasted in average 8 months. A second complete remission was obtained in seven patients. The overall survival after 2 years was 50% and 30% after 4 years. At the time of the investigation six patients are alive, three in complete remission and three in partial remission. Two patients are in partial remission after 6 years, one of these had additional therapy with alfa-interferon. Patients entering the study until 1982 also received transfer factor, an immune stimulating agent. Since in 1982 a double-blind study revealed no differences between patients given the active--and patients given the inactive medication, no new patients since then had transfer factor. BCPE compares favourable with other chemotherapeutic regimes. The data presented seem to justify the use of retinoids as a part of combination chemotherapy in mycosis fungoides.

Adult↗