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PubMed1977

Distribution of HLA antigens in patients with mycosis fungoides.

19 patients with plaque-style mycosis fungoides have undergone determination of HLA antigen distribution. Deviations from the expected distribution frequency occurred with the antigens of the W19 group comprising A29, W30, W31 and W32 and of B8 and A1. The W19 group deviation was statistically significant (chi2 = 9.7 p value less than 0.005). The significance of this observation in relation to reported immunological abnormalities in mycosis fungoides is discussed.

Adult↗

PubMedDate not supplied

[Results of photochemotherapy in a series of 47 patients with mycosis fungoides].

Oral photochemotherapy has been employed in the treatment of mycosis fungoides for more than 13 years. The Authors report their 9 year experience in a series of 47 patients affected by mycosis fungoides and treated by means of PUVA-therapy. All the patients were staged according to TNM system. 81.5% of 38 cases in the I stage of the disease obtained a complete remission, as did 50% of 6 cases in the II stage and one patient out of 3 in the III stage. Photochemotherapy seems to give satisfactory results mainly in the first stages of the disease. In fact, 45% of the patients who had obtained complete remission ot the end of the treatment were still free from disease after 5 years. Photochemotherapy, which in this series has not shown any important side effect, is a safe non-aggressive therapeutic choice in the management of the first stages of mycosis fungoides.

Adult↗

PubMed1979

The patch stage of mycosis fungoides. Criteria for histologic diagnosis.

It has long been claimed that a specific histologic diagnosis of mycosis fungoides cannot be made in the premycotic" or "eczematous" (patch) stage of the disease. Indeed, the histologic features of the premycotic lesions were constantly said to be those of "chronic non-specific dermatitis." We studied 46 biopsy specimens of patch lesions from patients in whom mycosis fungoides was unequivocally established by clinical events (i.e., concurrence or later development of typical plaque and/or nodular lesions) and indubitable histologic findings. We divided patch lesions into early nonatrophic patches and late atrophic ones. The early patches are considered to be evolving lesions of mycosis fungoides, whereas late patches represent resolving plaques of the disease. On the basis of this study, we concluded that histologic diagnosis can be made with near certainty in patch lesions of the disease. We found that the critical feature for histologic diagnosis of early and late patch lesions of mycosis fungoides is the presence of an increased number of mononuclear cells distributed singly or in small collections within an epidermis devoid of spongiotic microvesiculation. Other important features are lacunae surrounding intraepidermal mononuclear cells which gives them the appearance of "haloed cells." A sparse infiltrate of mononuclear cells is present around the blood vessels of the superficial, and sometimes the deep, vascular plexus. Atypical mononuclear cells are not necessary for the diagnosis of early patch lesions of mycosis fungoides, but they are found commonly in late patch lesions. Late atrophic patches show a thinned epidermis, loss of the usual configuration between rete ridges and dermal papillae, and coarse collagen throughout a thickened papillary dermis.

Adolescent↗

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↗

PubMed2003

Galectin-1-mediated apoptosis in mycosis fungoides: the roles of CD7 and cell surface glycosylation.

Sezary cells, the malignant T cells in mycosis fungoides/Sezary syndrome, resist a variety of apoptosis-inducing agents, a feature that contributes to the poor response to therapy in mycosis fungoides. Galectin-1 is a mammalian lectin that triggers T cell apoptosis. For T cells to be susceptible to galectin-1-induced apoptosis, the T cells must express specific glycoprotein receptors, such as CD7, that bear the specific oligosaccharides recognized by galectin-1. Because Sezary cells are characteristically CD7(-), lack of CD7 expression has been proposed to render Sezary cells resistant to galectin-1-induced death. However, the role played by aberrant cell surface glycosylation in resistance of Sezary cells to galectin-1 has not been examined. In this study, we demonstrated abundant galectin-1 in mycosis fungoides skin lesions, indicating that Sezary cells are exposed to galectin-1 in vivo. To determine specific characteristics of Sezary cells that contribute to galectin-1 resistance, we assessed CD7 expression and cell surface glycosylation of Sezary cells in mycosis fungoides lesions and of four Sezary T cell lines. Sezary cells in primary lesions and Sezary T cell lines demonstrated a characteristic "glycotype" with sialylated core 1 O-glycans that promote galectin-1 resistance. Expression of CD7 was necessary but not sufficient for galectin-1-induced death of Sezary cell lines. In addition, CD7(-) Sezary cell lines, and Sezary cells within mycosis fungoides lesions, expressed galectin-1, whereas CD7-positive Sezary cell lines did not express galectin-1. We propose that both loss of CD7 expression and altered cellular glycosylation contribute to apoptosis resistance of malignant T cells in mycosis fungoides.

Antigens, CD7↗

PubMed2005

A retrospective study of the probability of the evolution of parapsoriasis en plaques into mycosis fungoides.

Parapsoriasis en plaque has been suggested to be an early manifestation of mycosis fungoides (cutaneous T-cell lymphoma). We explored the disease course of patients with small plaque or large plaque parapsoriasis in a 26-year retrospective cohort analysis of 105 parapsoriasis patients, who were clinically and histopathologically followed up in Helsinki and Tampere University Hospitals. Eventual later cancers of these patients were verified from the Finnish Cancer Registry. In the small plaque parapsoriasis group, 7 patients (10%) and in the large plaque parapsoriasis group 12 patients (35%), developed histologically confirmed mycosis fungoides during a median of 10 and 6 years, respectively. No significant differences were found regarding the risk of developing mycosis fungoides or the tendency to remission in patients treated with or without phototherapy. Our results show that not only large plaque parapsoriasis, but also small plaque parapsoriasis, as currently defined in textbooks, can progress to mycosis fungoides. The benefits of phototherapy are equivocal in parapsoriasis treatment as far as progression to cancer is concerned.

Adult↗

PubMed1984

[Association of mycosis fungoides and Hodgkin's disease].

A 69-year-old man developed a Hodgkin's disease 2 years after he started a mycosis fungoides. He presented cutaneous plaques of mycosis fungoides. The first signs of Hodgkin's disease was acquired ichthyosis and loss of weight. Echotomography of the abdomen showed retroperitoneal nodes. A laparotomy was performed and the histopathologic examination of the lymph nodes revealed a Hodgkin's disease type 2 (sclero-nodular). The liver and the bone marrow were involved. A chemotherapy was completed but the patient died 10 months later. The review of the literature showed 24 patients with Hodgkin's disease and mycosis fungoides or Sézary syndrome. Relation between mycosis fungoides, Hodgkin's disease and lymphomatoid papulosis are discussed.

Aged↗

PubMed1985

Remission in skin infiltrate of a patient with mycosis fungoides treated with cyclosporine.

A 57-year-old woman with mycosis fungoides that had failed to respond to cytotoxic chemotherapy was treated with cyclosporine. Mycosis fungoides and Sézary syndrome are disorders of helper T cells. Cyclosporine is a fungal endecapeptide of novel chemical structure that causes preferential inhibition of T helper cells. Because of this in vitro inhibition of T helper cells, we used cyclosporine to treat a patient who had mycosis fungoides that was refractory to cytotoxic combination chemotherapy. With cyclosporine administered initially as an intravenous infusion and orally after 10 days, there was immediate improvement in the patient's symptoms. This subjective improvement was accompanied by a decrease in her skin infiltration, noted on physical examination and microscopically. Despite continued administration of the cyclosporine, symptoms recurred after 3 1/2 months of therapy.

Cyclosporins↗

PubMed1997

Analysis of V(H) genes rearranged by individual B cells in dermal infiltrates of patients with mycosis fungoides.

In patients with cutaneous T cell lymphomas such as mycosis fungoides B cells can frequently be detected in the lymphocytic dermal infiltrate. To analyse their immunoglobulin heavy chain gene repertoire, single B cells were obtained from tissue sections of two typical patients with mycosis fungoides using hydraulic micromanipulation followed by specific amplification of the respective gene segments by single-cell polymerase chain reaction (PCR) technique. A total of 21 V(H)DJ(H) genes was sequenced. From each individual B cell a single productive V(H)DJ(H) rearrangement was obtained. There was no clonal relationship detected between any of these rearrangements suggesting polyclonality of the infiltrating B cells. The representation of V(H) families was in accordance with the germ-line complexity. A remarkably high number of V(H) genes (5/13 in patient 1; 3/8 in patient 2) was completely or nearly germ-line-identical. Five of seven V(H)4 family genes were nearly unmutated. On the other hand, most of the V(H)3 gene family members were somatically mutated in an antigen-driven manner. The proportion of germ-line-identical V(H) genes, the usage of individual V(H), D, J(H) gene elements, and the pattern of somatic mutations found in the B cells infiltrating skin lesions of patients with mycosis fungoides resembles the peripheral blood repertoire, suggesting a bystander role of these cells.

Amino Acid Sequence↗

PubMed2003

Mycosis fungoides responding to systemic itraconazole.

Mycosis fungoides is a form of cutaneous lymphoma with a typically indolent course. We describe an unexpected, repeated response to a systemic antifungal agent, itraconazole. The mechanism of action is speculative but itraconazole has marked immunosuppressive activity on T-lymphocyte proliferation in vitro.

Administration, Oral↗

PubMed2005

Treatment with IFN-gamma increases serum levels of Th1 chemokines and decreases those of Th2 chemokines in patients with mycosis fungoides.

BACKGROUND: Interferon-gamma (IFN-gamma) is used for the treatment of mycosis fungoides, which is a Th2 neoplasm with elevation of serum Th2 chemokines. Although therapeutic effectiveness of IFN-gamma is caused at least partly by augmented activity of cytotoxic T cells against the tumor cells, its modulation on chemokine production remains unknown. OBJECTIVE: Alterations in the serum levels of Th1 chemokines, IP-10 and MIG, and Th2 chemokines, TARC and MDC, were examined in mycosis fungoides patients treated with recombinant IFN-gamma. METHODS: Four patients with mycosis fungoides received intravenous injections of IFN-gamma for 14 or 28 days. On day 0, 7, 14, and 28, sera were obtained from the patients, and the concentrations of TARC, MDC, IP-10, and MIG were measured by ELISA, along with the percentages of peripheral blood Th1 and Th2 cells. RESULTS: Whereas the levels of TARC and MDC were decreased by IFN-gamma treatment, those of IP-10 and MIG were increased. In particular, the increment of MIG was remarkable. No substantial change of Th1 or Th2 cell number was observed. CONCLUSION: In IFN-gamma treatment as well as other therapies, TARC may serve as a marker for the disease activity of mycosis fungoides. The dramatic elevation of MIG by IFN-gamma suggests the strong dependency of MIG production on IFN-gamma and the participation of MIG in skin-infiltration of tumoricidal cytotoxic T cells.

Aged↗

PubMed2003

[Long term results of total skin electron beam therapy (TSEBT) in the treatment of mycosis fungoides].

BACKGROUND AND OBJECTIVE: Total skin electron beam therapy (TSEBT) is an important therapeutic option for the treatment of mycosis fungoides. The aim of this study was to find out long term results in 10 patients with the clinical and histological diagnosis of mycosis fungoides. PATIENTS AND METHODS: Between 1982 and 1997 we performed a total skin electron beam therapy in 10 patients. One patient was in stage I A; 5, in stage I B; 3, in stage II B; and one, in stage IV A. The indication for TSEBT was disease progression in spite of PUVA therapy. The doses of electron beam therapy were 30- 36 Gy at 5-6 MeV. In 7 of 10 cases additional local electron beam therapy was given, because individual lesions persisted or recurred after a short time. RESULTS: Complete remission was achieved in 5 patients. The follow- up has been up to 10 years. One patient died of systemic lymphoma 10 years after electron beam therapy. Three patients died of illnesses unrelated to mycosis fungoides. One patient developed a high grade T-cell lymphoma. CONCLUSIONS: Total skin electron beam therapy is a very effective, but technically difficult therapy for mycosis fungoides, especially in stage I B and II B. Particularly interesting has been the long duration of complete response of three patients, who had follicular mucinosis preceding their mycosis fungoides.

Adult↗

PubMed1992

Infections complicating mycosis fungoides and Sézary syndrome.

OBJECTIVE: To determine, in patients with mycosis fungoides and Sézary syndrome, the incidence of infections, the importance of nosocomial infections, and the epidemiologic factors associated with cutaneous and visceral infections. DESIGN AND SETTING: Retrospective inception cohort study at a university medical center referral clinic. PATIENTS: Three hundred fifty-six patients with mycosis fungoides or Sézary syndrome. MAIN OUTCOME MEASURES: Incidence rates for specific infections, and multivariate risk ratios for demographic and clinical factors associated with infection. RESULTS: Cutaneous bacterial infection was most common (17.0 infections per 100 patient-years), followed by cutaneous herpes simplex virus and herpes zoster virus infection (3.8 infections per 100 patient-years), bacteremia (2.1 infections per 100 patient-years), bacterial pneumonia (1.7 infections per 100 patient-years), and urinary tract infection (1.4 infections per 100 patient-years). Twenty-seven percent of herpesvirus infections disseminated on the skin but none disseminated to internal organs. Pneumonia or bacteremia was present in 88% of patients who died of infection. Only three patients had invasive fungal or protozoal infection. Nosocomial infections accounted for 19% of cutaneous bacterial infections, 59% of bacteremias, 62% of pneumonias, and 88% of infections leading to death. By logistic and Cox regression, the presence of extracutaneous involvement with lymphoma was the most important independent risk factor for recurrent bacterial skin infection (risk ratio [RR], 12; 95% confidence interval [CI], 1.2 to 120), disseminated herpesvirus infection (RR, 28; 95% CI, 2.7 to 290), bloodstream infection (RR, 5.5; 95% CI, 1.7 to 18), and death from infection (RR, 15; 95% CI, 3.6 to 64). CONCLUSIONS: Community-acquired bacterial skin infections are a common cause of morbidity in patients with mycosis fungoides and Sézary syndrome but are usually treated without hospital admission. Bacteremia and pneumonia, which are usually nosocomial, are the major infectious causes of death. Advanced disease stage, independent of corticosteroids and other therapies, is the most important risk factor for both cutaneous and systemic infections.

Adolescent↗

PubMed1979

Mycosis fungoides: clinical and therapeutic review.

Mycosis fungoides is a cutaneously-derived malignant lymphoma which, without effective treatment, may run an unrelenting, rapidly lethal course. Often beginning as a nonspecific skin eruption, easily misdiagnosed as a common eczema or psoriasis, the disease progresses through a plaque stage to the tumor stage with the appearance of the mushroom-like growths for which the entity was originally named. Because of the dismal outlook in uncontrolled cases, newer, more aggressive approaches to therapy are being developed and are being instituted earlier in the course of the disease.

Humans↗

PubMed1996

Multiparametric image cytometry in mycosis fungoides.

Eighteen cases of early mycosis fungoides were compared with 18 cases of eczematous dermatitis by multiparametric image cytometry. A minimum of 100 lymphocytes was measured in each case. A large number of measurements was acquired for each lymphocyte, characterizing nuclear DNA content, area, shape, and texture. There were significant differences between the two groups, especially in nuclear DNA content and texture. These differences allowed the two groups of nuclei to be distinguished with 78% accuracy. The two groups of lesions were distinguished with 94% accuracy, using neural network analysis.

Cell Nucleus↗

PubMed1977

Scandinavian mycosis fungoides study group.

A multicenter study group on Mycosis Fungoides in Denmark, Norway and Sweden has been active since 1974. Staging procedures based on a 5 stage-system have been established, and treatment protocols have been worked out. Topical nitrogen mustard with and without toleranceinduction has been applied on stage II, and Bleomycin has been randomized against Bleomycin and Methotrexate in stage, III, IV, and V. Protocols containings PUVA are now activated, with PUVA given in combination with Cyclophosphamide for extracutaneous forms of Mycosis Fungoides.

Humans↗

PubMed1994

Interferon-alpha (INF-alpha) and etretinate in the treatment of mycosis fungoides.

A combination therapy of mycosis fungoides (MF) with interferon-alpha (INF-alpha) and etretinate was reported. A 53-year-old patient suffering from MF was treated with intramuscular administration of INF-alpha at a dosage of 9 x 10(6) IU daily; skin lesions poorly responded to the treatment. After oral etretinate with a dosage of 50 mg daily was combined with INF-alpha, marked clinical improvement was seen in skin lesions with minor side effects. INF-alpha in combination with etretinate can have beneficial effects in the treatment of MF.

Administration, Oral↗