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PubMed1999

Twenty-year trends in the reported incidence of mycosis fungoides and associated mortality.

OBJECTIVES: Patterns of mycosis fungoides incidence and associated mortality in the United States were evaluated. METHODS: Data were taken from the Surveillance, Epidemiology, and End Results cancer registry program and the National Center for Health Statistics. RESULTS: The incidence rate from 1973 through 1992 was 0.36/10(5) person-years. The age-adjusted incidence rate ratio of Blacks to Whites was 1.7; that of Asians to Whites was 0.6. There was no evidence of increasing incidence rates during the period 1983 through 1992. Mortality rates declined steadily from 1979 to 1991 and were less heterogeneous geographically than incidence rates. Mortality rate patterns with age, sex, and race were similar to the corresponding incidence patterns. CONCLUSIONS: The incidence rate of mycosis fungoides has stabilized and the mortality rate has declined. For unknown reasons, the disorder varies greatly among demographic and geographic subgroups.

Adolescent↗
PubMed1979

Dose considerations in total skin electron irradiation for mycosis fungoides.

Two hundred patients with generalized cutaneous mycosis fungoides were treated with total skin electron irradiation (TSEI) in the Lahey Clinic Radiotherapy Department-MIT High Voltage Research Laboratory between 1964 and 1973. None of the patients had any clinical evidence of extracutaneous disease at presentation. The 3-year absolute survival rate was 54% (107 patients). Analysis of these 107 long-term survivors evaluated the relation between incidence of generalized cutaneous recurrence and total treatment dose given during the initial course of TSEI. Results indicated that in patients with erythroderma, the need for a second course of TSEI was inversely related to the total dose given during the first course. However, the curve seemed to flatten at about 1,500 cGy (rad). In plaque disease, the percentage of patients who received repeated courses of TSEI seemed to be relatively constant independent of total dose given during the initial course (65%-80%). In patients with tumor lesions, the incidence of generalized cutaneous recurrence was directly proportional to total dose. The optimal dose of TSEI for patients with mycosis fungoides may vary depending on types of skin lesions. Total dose during the initial course of TSEI should be kept relatively conservative, particularly in patients with generalized plaques where relapse rate is high allowing reserve of normal tissue tolerance for further TSEI when indicated.

Dermatitis, Exfoliative↗
PubMed1994

Presence of Epstein-Barr virus in cutaneous lesions of mycosis fungoides and Sézary syndrome.

It has been suggested that prolonged antigenic stimulation contributes to the development of epidermotropic cutaneous T cell lymphoma (CTCL), mycosis fungoides and Sézary syndrome, characterized by a cutaneous infiltration of proliferating helper T cells. Since Epstein-Barr virus (EBV) antibodies were increased in CTCL sera, we investigated a possible etiologic role for EBV in epidermotropic CTCL by looking for the EBV genome in 25 cutaneous biopsies of mycosis fungoides or Sézary syndrome and 12 reactional inflammatory skin lesions. The use of a non-isotopic in situ hybridization procedure based on the detection of Epstein-Barr encoded RNAs with biotinylated oligonucleotide probes (EBER) revealed 32% of the lesions with CTCL to be positive for EBV (3 in dermis, 3 in epidermis, 2 both in dermis and epidermis), as compared to no detection of the EBV genome in the reactional inflammatory skin lesions. Moreover, a combined hybridization (EBER probe) and immunochemistry technique (anti-CD3 or anti-Kil monoclonal antibody) permitted the identification of EBV in T cells of dermis and in keratinocytes, respectively. The identification of EBV in epidermotropic CTCL suggests that this virus could play a role in the development of these CTCLs, either as an etiological agent or more probably as a chronic activating agent. Indeed, the infection of keratinocytes by EBV could activate them and so induce the production of in situ cytokines (IL1a, IL6, TNFa) playing a role in the development of tumoral infiltrate.

Adult↗
PubMed1981

Megavolt electron irradiation for localized mycosis fungoides.

A retrospective analysis was carried out to evaluate the effectiveness of small field megavolt electron irradiation for localized mycosis fungoides. Only local field electron beam therapy was employed for limited disease reserving total skin electron irradiation for multiple lesions or diffuse disease covering at least 25 per cent of the entire body surface. Of the 14 patients with limited disease treated between 1964 and 1973 with the local field technique, 10 patients (71 per cent) are alive without evidence of disease at a minimum of 5 years. In contrast, of 200 patients with extensive cutaneous disease who received total skin electron irradiation, only 16 (8 per cent) were considered cured. It is concluded that early localized mycosis fungoides is potentially curable, and that limited field electron beam therapy with a relatively low total dose is adequate to obtain excellent response.

Humans↗
PubMed2006

Treatment of mycosis fungoides with denileukin diftitox and oral bexarotene.

Cutaneous T-cell lymphomas, including mycosis fungoides and Sezary syndrome, are often responsive to treatment, but current therapies have not been shown to increase survival, and in advanced stages, durable remissions are hard to achieve. We present a patient who was initially misdiagnosed with psoriasis and, 16 years later, was diagnosed with mycosis fungoides. Denileukin diftitox was used as a tumor debulking agent to give a partial response that was further improved with a combination of systemic interferon/oral bexarotene and skin-directed psorlen plus UV-A. The purpose of this case report is to show the value of sequential combination therapy for improving overall response.

Administration, Oral↗
PubMed2006

Mycosis fungoides with coccidioidomycosis.

Mycosis fungoides is an indolent, epidermotropic, cutaneous T-cell lymphoma of helper/memory T cells that presents as heterogeneous, papulosquamous patches, plaques, and tumors. We present a patient with mycosis fungoides and infection with Coccidioides immitis of the skin, which has not been previously reported.

Adenocarcinoma↗
PubMed2006

[Imiquimod in treatment of palpebral mycosis fungoides].

CLINICAL CASE: Our patient was a 37-year-old male with palpebral mycosis fungoides, the most common T-cell cutaneous lymphoma. He had been treated previously with topical corticosteroids. We treated him with Imiquimod cream (5%) three times a week. DISCUSSION: A clinical improvement was obtained with this drug, so we believe immuno-modulating agents need to be considered in the treatment of palpebral mycosis fungoides, especially because of its capacity of being a reflection of leukaemia.

Adult↗
PubMed1999

Follicular mycosis fungoides: presentation of a case and review of the literature.

Follicular mycosis fungoides is a rare variant of mycosis fungoides (MF). Structural-wise there are several acneiform lesions made up of comedones, cysts and hyperkeratosis. The main histological finding is atypical lymphocytic infiltration around follicular structures, without epidermotropism. The association with follicular mucinosis is widely discussed in the literature. We report a case of follicular (MF) and review the cases published to date.

Humans↗
PubMed1979

Once weekly total-skin electron-beam therapy for mycosis fungoides: 7 years' experience.

A total of 115 patients with mycosis fungoides were given total-skin electron-beam therapy (TSEB), utilizing 3.5-mev electrons at doses of 400 rads to the entire skin surface once a week for 6--8 consecutive weeks. Prompt relief of symptoms and regression of lesions were observed in all patients. Of the 81 patients at risk for 12--91 months (median, 24 months) following TSEB, initial unmaintained remission lasted 6--69 months (median, 19 months). No untoward immediate or late effects have been noted in the bone marrow or normal skin which was irradiated. The duration of remission following TSEB correlated well with lymphocyte responsiveness to various mitogens and antigens, but not with the initial response. Thymic hormone factor levels (Facteur Thymic Serique) were elevated in the majority of these patients with mycosis fungoides.

Adult↗
PubMed1979

Electron-beam therapy for mycosis fungoides: the Stanford University experience.

The use of high-dose electron-beam therapy for mycosis fungoides at Stanford University is reviewed. Since 1966, 140 patients have been treated in this fashion. Their clinical characteristics including initial extent of disease are reviewed. The results of routine staging studies are examined. Eighty-four percent of the patients achieved an initial complete remission; this was inversely related to the initial extent of skin involvement. The overall survival rate was 46% at 10 years with the major prognostic factor being the initial extent of skin involvement. Other factors which had an influence on the patient's progrnosis included the presence of palpable adenopathy, the patient's age, the achieving of an initial complete remission, the initial dose of electron-beam therapy, and the treatment with adjuvant topical mechlorethamine. The development of a rationale for the treatment of patients with all stages of mycosis fungoides based upon these clincal observations is discussed.

Adult↗
PubMedDate not supplied

[The ultrastructural characteristics of the early diagnosis of mycosis fungoides].

As a result of studies made (biopsy specimens of damaged skin from seven patients presenting with the erythrodermic stage of mycosis fungoides) a conclusion was reached that at the given stage the following two processes take place: (1) a rise in the proliferative activity of keratinocytes and (2) disturbances their differentiation. These ultrastructural manifestations can serve as criteria for a well-timed diagnosis of mycosis fungoides to be made.

Biopsy↗
PubMed1975

Treatment of mycosis fungoides with topical nitrosourea compounds: Further studies.

Twenty-six patients with mycosis fungoides were treated topically with three nitrosourea compounds: carmustine (BCNU), lomustine (CCNU), and 1-methyl-1-nitrosourea. A high percentage experienced good to excellent results. Remissions following treatment of individual lesions varied from one month to at least three years. Remissions following total body surface treatment varied from two weeks to at least four months. Two of 13 patients treated over the entire body suffered temporary bone marrow depression, indluding one with severe pancytopenia. This toxic effect was attributed to lomustine and was not seen in patients treated with carmustine alone. Thirteen patients highly allergic to mechlorethamine hydrochloride showed no cross-sensitivity to nitrosourea compounds. A primary irritant dermatitis occurred in about one half of the patients and telangiectasia in two. Two patients developed hypersensitivity to nitrosourea compounds. Carmustine is the preferred nitrosourea compound for topical therapy of mycosis fungoides.

Administration, Topical↗
PubMed2002

[Mycosis fungoides. Study of a series of 11 Tunisian cases].

Mycosis fungoides is an epidermotropic cutaneous T lymphoma. It's a non Hodgkinian lymphoma. We report the results of a retrospective review of 11 mycosis fungoide seen during 22 years. The frequency of MF was about 39.3% among all cutaneous lymphoma. Six patients were male and 5 were female; the mean age was about 56 years. Mean delay between diagnostic and the first manifestation was about 25 months. All patients had the progressive form: 4 had infiltrate plaques and 7 were at the tumoral phase. Lymph nodes and medullar metastases were noted respectively in 1 and 2 cases. Treatment was mono or polychemotherapy associated in 6 cases with topical drug. Three patients died of their diseases According to our experience and after reviewed the literature, we notice that our patients are slightly younger without male predominance. The diagnostic was done tardily and this may explain the pejorative prognostic.

Administration, Topical↗
PubMedDate not supplied

[Immunological studies on patients with mycosis fungoides].

Recently, much has been published on the immunological status of patients affected with various lymphomas. In the particular case of Mycosis fungoide, there was no general agreement on the immunological status of the corresponding patients. In fact, López Borrasca et al, found severe depression of cellular immunity in such patients. On the contrary, Blaylock and Clendenning found very little change in cellular immunity, but a very high serum-IgA. We want to offer our experience on this problem with the immunological survey of four patients with the Alibert-Bazin-form of Mycosis fungoide. The following tests were performed on each patient: a) Intracutaneous test with candidina, PPD and other bacterial antigens. b) Sensitization to a concentrate solution of dinitrochlorobencene (DNCB). c) Lymphocyte Transformation Test (LTT), with phytohemagglutinin as mitogen. d) Quantitative determination of IgG, IgM, IgA and beta1C, with the radial immunodiffusion technique (Mancini et al.). e) Agar immunoelectrophoresis. The following results were obtained: 1) The cellular immunity was markedly depressed in the four patients when any of a, b or c-test was performed. 2) All the patients showed very high levels of serum IgA, 150% higher than control. The reason for this is unknown. On the contrary, IgG in serum was less elevated and IgM and beta1C serum levels were normal. 3) No monoclonal bands were found in any case (immunoelectrophoresis). 4) No definite conclusions could be reached due to the limited number of cases, but the uniformity of results should encourage to carry this work further.

Angioedema↗
PubMed1975

[Mycosis fungoides].

The classical characteristics of Myocsis Fungoides are discussed. 1) The clinical characteristics of the three forms: tumour from the start, erythrodermic and polymorphic forms of Alibert-Buzin. 2) Histological characteristics : dense dermal infiltrates or epidermotropic infiltration, or around a more or less malignant histiocytic reaction, there develops a host reaction, resembling a granuloma. During the course of the disease, one may observe lymphocyte depletion, whilst delayed hypersensitivity tests become negative. The various types of lymphadenopathy are analysed, the possibility of visceral lesions confirmed. However, knowledge of Sezary's syndrome makes his concept of the disease doubtful. A pathological picture similar to Mycosis Fungoides may be observed during Sezary's syndrome, with circulating giant cells. The presence of small Sezary cells, Lutzner-Flandrin variety, is common in the dermal infiltrate, and even more common in the lymph node visceral infiltrates of Mycosis Fungoides. Thus, one is led to the concept according to which the primary phenomenon is probably pathological proliferation of abnormal T lymphocytes (large and small Sezary cells), the histiocytic proliferation would then simply be a secondary reaction. This theory is then discussed.

Adult↗
PubMedDate not supplied

Ichthyosiform mycosis fungoides.

Malignancy-associated acquired ichthyosis is well known, but the ichthyosiform subset of mycosis fungoides (MF) is rarely reported. We report on two patients with a clinical presentation for whom diagnosis of mycosis fungoides was established on histological grounds. In both cases, long term remission was obtained with non aggressive therapies. This rare condition must be added to newly described forms of MF with epidermal hyperplasia such as keratosis lichenoides chronica like MF and pilotropic MF.

Aged↗
PubMed1992

Human T-cell leukemia virus type I or a related retrovirus in patients with mycosis fungoides/Sézary syndrome and Kaposi's sarcoma.

Antibodies reactive with human T-cell leukemia virus type I (HTLV-I) proteins p19, p24, gp46, p56, and gp68 were detected in four of 27 patients with mycosis fungoides/Sézary syndrome (MF/SS) and one patient with Kaposi's sarcoma using radioimmunoprecipitation and Western blot analysis. Seroreactivity patterns to HTLV-I proteins of MF/SS sera were indeterminate or limited in comparison with sera of patients with adult T-cell leukemia/lymphoma. HTLV-I gag- and tax/rex-specific DNA was demonstrated in peripheral blood from three of the MF/SS patients and from the patient with Kaposi's sarcoma by the polymerase chain reaction. HTLV-I-specific DNA sequences were not detected in a cohort of seven seronegative MF/SS patients. The frequency of HTLV-I infection was four of 27 or 14.8% among the MF/SS patients, which is several hundredfold higher than in normal blood donors. The present data suggest a possible association of HTLV-I or a related retrovirus with mycosis fungoides/Sézary syndrome and Kaposi's sarcoma.

Adult↗
PubMed1977

Cytophotometric studies on mycosis fungoides and other cutaneous reticuloses.

Feulgen-DNA cytophotometry was carried out in skin imprint preparations of patients with mycosis fungoides, reticulum-cell sarcoma, lymphomatoid papulosis and Sézary's syndrome. These patients proved to have aneuploid and polyploid DNA histograms. In tumour stage of mycosis fungoides a more bimodal distribution of the DNA values was found. Of 79 cases of suspected malignant reticulosis of the skin, 32 showed an abnormal DNA histogram with 5 percent or more tetraploid and hypertetraploid cells. Thirty of these patients developed a malignant skin reticulosis during the 4-year follow-up period. It is concluded that DNA cytophotometry has value as a supplement to routine morphological histopathology in malignant cutaneous reticulosis.

DNA, Neoplasm↗