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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↗
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↗
PubMed1991

[Mycosis fungoides. Clinical characteristics and treatment of 6 patients].

Mycosis fungoides is a low grade malignant cutaneous T-cell lymphoma. It is a rare disorder where diagnosis may be difficult to establish in early stages. Usually several years may pass between onset of disease and confirmation of the diagnosis, which should be confirmed histopathologically. Prognosis is difficult to predict and depends on whether mycosis fungoides is limited to the skin or has spread to lymph nodes or viscera. Based on the confirmation of six new cases of mycosis fungoides during the last 18 months by the Department of Dermatology, University Hospital of Tromsø, we discuss clinicopathological findings and alternative methods of treatment.

Aged↗
PubMed1990

Coexisting follicular mucinosis and mycosis fungoides--a case report.

This is a case report of a patient with mycosis fungoides coexisting with follicular mucinosis. The simultaneous occurrence of these two conditions has been documented. There is controversy as to whether mycosis fungoides in patients with follicular mucinosis arises de novo or whether follicular mucinosis can evolve into mycosis fungoides. The relationship between the two conditions is discussed.

Adult↗
PubMed1990

[The cytotoxic effect of the thrombocytes from patients with mycosis fungoides and Kaposi's sarcoma].

The platelet cytotoxic action on tumor cells was studied in patients with mycosis fungoides and Kaposi's sarcoma. The findings evidence, that along with the known populations of cytotoxic effector cells, such as lymphocytes and monocytes, platelets may destroy tumor cells as well. Platelet ability to induce tumor cell lysis is essentially increased in mycosis fungoides patients treated according to scheme B. The data indicate that platelets from patients with mycosis fungoides and Kaposi's sarcoma are characterized by cytotoxic activity against AKL continuous tumor target cell line.

Adolescent↗
PubMed1990

Depressed lymphokine activated killer cell activity in mycosis fungoides. A possible marker for aggressive disease.

Peripheral blood mononuclear cells from 24 patients with mycosis fungoides were used to generate lymphokine activated killer (LAK) cells in vitro by culturing with recombinant interleukin 2. Patients with stage la mycosis fungoides were capable of generating normal levels of LAK cell activity, while patients with more active disease (stages IB to IV) had depressed LAK activity. The ability of these patients' cells to respond in a proliferation assay to various mitogens was similar to that of controls, with the exception of patients in the terminal phase of their illness. Patients with active disease who were unable to generate LAK activity were capable of responding in a proliferation assay to interleukin 2. The results of this study suggest that depressed LAK cell activity in patients with mycosis fungoides may serve as an indicator of a more aggressive disease state.

Adult↗
PubMed1990

[The applied significance of a study of the biochemiluminescence of the blood serum in patients with mycosis fungoides].

Analysis of chemiluminescence levels of blood sera from 64 patients with mycosis fungoides and 44 ones with nontumor dermatoses (eczema, psoriasis, secondary erythroderma) has helped specify some aspects in the pathogenesis of mycosis fungoides. Basing on the findings of this analysis, the authors have developed an auxiliary method for differential diagnosis of mycosis fungoides and nontumor dermatoses, whose clinical patterns are similar, and a method for assessing the tumor cell sensitivity to prescribed polychemotherapeutic complex and finding the optimal period between the courses of therapy.

Adult↗
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↗
PubMed1989

Plasma cells in the dermal infiltrate of mycosis fungoides are of polyclonal origin.

There are several reports on the occurrence of immunoglobulin-producing B-cell lymphomas in Sézary's syndrome and mycosis fungoides, which may be the consequence of the helper activity of the neoplastic T-cells. Therefore we investigated skin biopsies of 50 patients with mycosis fungoides regarding the presence of plasma cells and their immunoglobulin profile. Nine of these patients had plasma cell nests, most frequently located at the lower edge of the infiltrate. IgE was detectable consistently, and IgG, IgM and IgA could also be demonstrated in the majority of these cases; kappa- and lambda-chains were present in equal amounts. Our results demonstrate polyclonal activation of plasma cells in a subgroup of mycosis fungoides patients.

Humans↗
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↗
PubMed1989

[The significance of epidermal changes in the early diagnosis and development of mycosis fungoides].

In 47 patients with stage I mycosis fungoides, biopsy of skin has revealed substantial changes in epidermis acanthosis with confluent epidermal processes, focal dystrophy of basal cells, mitoses in various epidermal layers, parakeratotic foci without a granular layer. Histoautoradiographic and immunomorphological studies demonstrated that enhanced proliferative activity of keratinocytes that was more pronounced in stages I and II mycosis fungoides and impaired epidermal differentiation that was predominant in neoplastic stage III underlay the above changes. There was ultrastructural evidence for abnormal epidermal keratinization. It was proposed that epidermal abnormalities made an important contribution to the development of mycosis fungoides.

Fluorescent Antibody Technique↗
PubMed1989

[Total cutaneous electron beam therapy of mycosis fungoides].

Electron beam irradiation of the entire skin surface was used to treat 25 patients with mycosis fungoides from 1977 to January 1988. A plexiglas screen was used to reduce the energy of the 8 MeV beam of a Sagittaire linear accelerator to 4 MeV. A total dose of 30 Gy was delivered in 12 fractions over days. This series includes 17 men and 8 women with a mean age of 44 years (range 13-78 years) and a mean follow-up of 34 months (range 6-92 months). The following-up staging system was used: stage A: superficial lesions covering less than 50 p. 100 of the body surface; stage B: superficial lesions covering more than 50 p. 100 of the body surface; stage C: tumors of the skin, lymph nodes and/or visceral organs, Sezary's syndrome. All stage A patients achieved complete remission. One developed recurrent disease in a very limited area 17 months after radiation therapy. No stage A patient died of mycosis fungoides. 6/9 stage B patients achieved complete remission; 4 of these developed recurrent disease localized to the skin 6 to 13 months after electron therapy. These recurrences were controlled by topical nitrogen mustard, puva therapy or localized irradiation. 1 patient showed no response and died of cutaneous mycosis fungoides. 5/10 stage C patients obtained complete remission but all relapsed within a mean period of 7 months. 4/5 of the patients not responding to electron therapy died of their disease and one is alive 16 months after completion of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1978

Mycosis fungoides evolving to myelomonocytic leukemia.

Acute myelomonocytic leukemia, a type of leukemic conversion that may be a manifestation of mycosis fungoides, developed in two patients with fairly typical courses of mycosis fungoides. The cases of five other patients with myelogenous leukemia as the terminal event of mycosis fungoides have been reported in the literature. A relationship to therapy could not be established, although all patients had had irradiation or chemotherapy or both.

Female↗
PubMed1977

Immunocytological characterization of the mycosis fungoides tumour cell.

The immunocytological identification of the mycosis fungoides cell was carried out on cells extracted from the tumorous nodules of a patient suffering from typical mycosis fungoides. Various techniques, such as E and IgM-EAC rosettes and examination for surface membrane immunoglobulins, were performed on the peripheral blood cells and the tumour cell. Membrane staining with a specific anti-T lymphocyte serum conjugated with peroxidase confirmed the thymodependent origin of the mycosis fungoides cell. However, the immunolabelling (with Fab peroxidase conjugate was found constantly negative.

Humans↗
PubMed1978

Mycosis fungoides-like cells. Their presence in a case of pityriasic dermatitis with a comment on their significance as an indicator of primary T-cell dyscrasia.

A case of pityriasic dermatitis in which the histologic findings mimicked mycosis fungoides was examined ultrastructurally. Large numbers of mycosis-like cells were found in the dermis and within epidermal spongiotic vesicles. Such cells occur in the epidermal and dermal infiltrates of primary T-lymphocyte disorders, notably in mycosis fungoides, the Sézary syndrome, and parapsoriasis en plague. However, they have also been found in the dermal infiltrates of benign dermatoses, in some skin tumors, and occasionally in normal controls. They share ultrastructural features with transformed T lymphocytes. It is emphasized that cells with this morphology may be found in the skin in any condition involving T-cell transformation or dysplasia. The mere presence of cells with this morphology within skin infiltrates is insufficient evidence for the diagnosis of primary T-cell dyscrasia.

Diagnosis, Differential↗
PubMed1977

Defective monocyte chemotaxis in mycosis fungoides.

Monocyte-macrophage function was studied in 14 patients with histologically confirmed mycosis fungoides. Three component parts of the monocyte-macrophage system were examined. Monocyte bacterial phagocytosis and bacterial killing were normal. However, a defect in directional mobility (chemotaxis) was found in patients with mycosis fungiodes (P less than .0025) when compared to 35 healthy controls. Defective chemotaxis was present regardless of disease stage or therapy. This defect in monocyte chemotaxis represents a previously unrecognized immune deficiency and may help explain the frequent infections found in patients with mycosis fungoides.

Adult↗
PubMed1988

Mycosis fungoides in the United States. Increasing incidence and descriptive epidemiology.

The etiology of mycosis fungoides is obscure, and the risk factors for its occurrence are poorly documented. This investigation uses data from nine US population-based cancer registries to investigate the descriptive epidemiology of this disorder. From 1973 through 1984, 721 newly diagnosed cases of mycosis fungoides were reported to these registries (0.29 cases per 100,000 population per year). A dramatic increase in the incidence of mycosis fungoides was noted over the period of this study. The incidence was highest among the elderly. Blacks were twice as likely to be afflicted as whites, and the incidence among men was more than twice the incidence among women. The geographic variation in incidence was associated with several demographic variables, including population density, family income, and concentration of physicians. Analysis of mortality among these patients revealed no evidence of detection bias.

Adult↗
PubMed1986

[Treatment of symptomatic mucinosis follicularis in mycosis fungoides using fast electrons].

Mucinosis follicularis is a chronic type of dermatosis involving the sebaceous glands and outer root sheaths, which can be differentiated into primary and secondary types. The latter - also called symptomatic mucinosis follicularis - causes therapeutic problems because of the underlying malignant lymphoma. Successful treatment using electron beams supported by etretinate is described in detail in a 40-year-old man with symptomatic mucinosis follicularis based on plaque-type mycosis fungoides. After 6 months free of skin symptoms, the mycosis fungoides lesions recurred in the overlapping borders of the irradiation fields. These lesions were treated again with electron beams. A relapse after 32 months was controlled by PUVA. Electron beam therapy is recomended in the plaque type of mycosis fungoides, especially if PUVA has no effect for some reason.

Adult↗