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Adenosine deaminase activity in sera of patients with psoriasis, mycosis fungoides and adult T cell leukemia.

Adenosine deaminase activities in sera were measured in 18 psoriatic patients, 8 mycosis fungoides patients, and 9 patients with adult T cell leukemia. Adenosine deaminase activity in the sera of the psoriatic patients showed no significant increase. An elevated adenosine deaminase activity was observed in 7 of the 8 patients with mycosis fungoides and 8 of the 9 patients with adult T cell leukemia. After chemotherapy, adenosine deaminase activity in serum of acute adult T cell leukemia was reduced. Adenosine deaminase activity in the sera of 2 patients with smoldering adult T cell leukemia was more elevated, with exacerbation of the disease. It is difficult to grade the extension of the tumors in plaque stage mycosis fungoides and smoldering adult T cell leukemia. To know the progression of the disease is critical in determining its management. These results indicate that adenosine deaminase activity in serum is one of the reliable indicators for the grading of mycosis fungoides and adult T cell leukemia.

Adenosine Deaminase↗

Clinicopathological spectrum of mycosis fungoides type cutaneous T-cell lymphoma.

OBJECTIVE: To determine the clinical, histological, and immunophenotypic characteristics of mycosis fungoides type cutaneous T-cell lymphoma. DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: This study was conducted from January 2000 to December 2004 at the Department of Dermatology, Military Hospital and the Department of Dermatopathology, Armed Forces Institute of Pathology, Rawalpindi. MATERIALS AND METHODS: The medical case records of patients with mycosis fungoides diagnosed during the period of study were surveyed. Data was collected pertaining to patient s characteristics, clinical descriptions, histopathological features, immunophenotypic analysis and stage of disease at the time of diagnosis. RESULTS: A total of 33 cases of mycosis fungoides were diagnosed between the years 2000 and 2004. There were 24 male and 9 female patients with male to female ratio of 2.6:1 The age ranged from 24 to 68 years and the duration of disease prior to diagnosis varied between 2 to 36 months. The number of skin biopsies performed for definite diagnosis ranged from 01 to 5. The various clinical presentations recorded in these patients were hypopigmented patches in 7 (21.3%), infiltrated papules and plaques in 6 (18.2%), erythroderma in 5 (15.2%), psoriasiform lesions in 3 (9%), and nodular lesions in 3 (9%) patients. There were 2 (6%) cases respectively of noduloulcerative, ichthyosiform and poikilodermatous lesions, and 1(3%) case each of follicular, morphoea-like and purpuric skin lesions. The predominant histological features were lymphocytic infiltrate in the upper dermis, epidermotropism, haloed lymphocytes in epidermis, Pautrier s microabscesses, and interface dermatitis. The immunohistochemical studies (n=12) showed predominantly T helper cell immunophenotype (CD3+, CD45RO+) in 11(92%) cases and T suppressor cell immunophenotype (CD3+, CD8+) in 1(8%) patient. CONCLUSION: The mycosis fungoides type cutaneous T-cell lymphoma has a wide clinicopathological spectrum. In a clinically non-specific dermatosis, a high index of suspicion and a regular follow-up may eventually lead to the definite diagnosis.

Adult↗

Mycosis fungoides of the larynx: case report and review of the literature.

Mycosis fungoides is a rare cutaneous lymphoma. Dissemination to extra-cutaneous sites occurs at advanced stages of disease. Laryngeal manifestations of mycosis fungoides have been reported in only 13 cases in the available literature. We present a further calla of mycosis fungoides of the larynx at the terminal stage of disease with an additional manifestation in the left maxillary sinus and review the cases published to date. To our knowledge this is the first reported case of mycosis fungoides with laryngeal and paranasal sinus manifestation. Concluding from the present case and from literature therapy should be palliative to improve quality of life.

Aged↗

Granulomatous mycosis fungoides. Clinicopathologic study of two cases.

Granulomatous mycosis fungoides is a rare form of mycosis fungoides with controversial histogenesis. Early reports seemed to indicate a favorable prognosis for these patients. We report two cases of granulomatous mycosis fungoides, both of which had other unusual clinical features. The cases were studied with routine light microscopy, immunohistochemistry, electron microscopy, and gene probe studies. Despite some clinical and histopathologic similarities, the results of the immunohistochemical and molecular biologic studies were diverse. These results suggest that granulomatous mycosis fungoides does not define a single subset of cases, immunophenotypically or biologically.

Adult↗

Implication of the ras and myc oncoproteins in the pathogenesis of mycosis fungoides.

In the present work, we studied the expression of the c-myc oncoprotein p-62 and the ras oncoprotein p-21 in the dermal cellular infiltrate of paraffin embedded skin specimens, obtained from patients suffering from Mycosis Fungoides and Sezary syndrome. Nineteen specimens from early stage Mycosis Fungoides, nineteen from advanced stage Mycosis Fungoides and four from Sezary syndrome were included in the study. The oncoprotein detection was achieved immunohistochemically, using the mouse monoclonal antibody myc 1-9E10 and the rat monoclonal antibody Y13-259 for p-62 and p-21 respectively. Increased detection of both p-62 and p-21 in atypic lymphoid cells was shown in advanced stages of Mycosis Fungoides (third stage plaques and tumors) as compared to early stages (premycotic erythema, second stage plaques). In advanced stages, however, the percentage of P-62+ atypic cells proved to be higher than that of p-21+ atypic lymphoid cells. The implication of increased p-62 and p-21 oncoprotein expression in the process of lymphomagenesis in cutaneous T-cell lymphomas is discussed.

Antibodies, Monoclonal↗

Intraepidermal but not dermal T lymphocytes are positive for a cell-cycle-associated antigen (Ki-67) in mycosis fungoides.

The Ki-67 antibody, which reacts with nuclei of actively proliferating cells, was used in an immunohistochemical study to determine if there was any difference between T cells located in the epidermis rather than the dermis, in mycosis fungoides. In 12 of 14 cases of patch/plaque stage mycosis fungoides, the epidermal T cells were Ki-67 positive, while the dermal T cells were Ki-67 negative in all cases. Both epidermal and dermal T cells belonged primarily to the memory-versus-naive subset. The intraepidermal Ki-67-positive T cells were slightly larger than the dermal Ki-67-negative cells and could be easily distinguished from occasional basal keratinocytes that were also Ki-67 positive. We conclude that dermal T cells, despite expressing HLA-DR and a memory phenotype, are essentially in a resting (Go or noncycling state) in mycosis fungoides. Furthermore, it appears that the movement of T cells into the epidermal compartment is associated with activation and entry into the cell cycle. Such intraepidermal activation may lead to lymphokine release, and play an important pathophysiologic role in mycosis fungoides.

Antigens, Differentiation↗

[Mycosis fungoides. Results of helioclimate therapy in high mountains (Davos, 1,560)].

The efficacy of topical steroids, chemotherapy, photochemotherapy, grenz-ray therapy and electron beam therapy has already been established in the treatment of mycosis fungoides. In addition, the results of dermatological climatotherapy in the high Alpine region (Davos, 1,560 m) demonstrate that natural sun irradiation in this particular climate is very effective in treating mycosis fungoides. A total of 63% out of 128 treatment cycles of 84 patients suffering from mycosis fungoides went into remission, which lasted a maximum of at least 13 months. The best results are obtained in the early stages of mycosis fungoides and if climatotherapy lasts long enough and is repeated.

Altitude↗

Mycosis fungoides. Topical use of nitrogen mustard in recurrent cases.

The management of the patient with mycosis fungoides requires a variety of therapeutic modalities depending on the stage of the disease. Topically applied nitrogen mustard in the early stages of the disease has a beneficital palliative effect. The effects of nitrogen mustard paintings in the later course of the disease have not been previously reported. In the present study, topically applied nitrogen mustard solution was used to control recurrences of mycosis fungoides following electron beam therapy in 11 patients. Each patient received whole body applications of freshly prepared 10 mg per 50 ml solution of mechlorethamine hydrochloride (a nitrogen mustard) in water daily for seven days. In all patients pruritus disappeared within the first week and ulcers and plaques improved or disappeared in two to four weeks. The seven-day courses of mechlorethamine paintings were repeated as recurrences were noted. Mycosis fungoides was controlled by this therapy for periods ranging up to 15 months. Absence of systemic toxicity, a low incidence of cutaneous irritation and application of the treatments at home make topical nitrogen mustard a useful adjunct in the management of the late stages of mycosis fungoides.

Adult↗

[Change in the turnover of HLA ABC molecules in circulating T lymphocytes in mycosis fungoides].

The turnover of HLA ABC molecules at T and B lymphocyte surface was analyzed in five cases with mycosis fungoides and six healthy controls. The patients had only skin lesions and are staged from T1 to T3 and No, Bo and Mo. The turnover was analyzed by mean of the decrease of the lysis by complement on sensitisized cells with anti HLA ABC antibodies that were incubated for progressive times at 37 degrees C. The results show a largest turnover for the HLA ABC molecules all T cells surface from mycosis fungoides that was significant (PWilcoxon less than 0.01). The turnover of B cells surface was not different from mycosis fungoide and healthy controls. The observed phenomenon was not specific for mycosis fungoide or T cells because previously has been shown in others lympho-proliferative and autoimmune diseases. The authors suggest that the analyzed T lymphocyte, morphologically normal is functionally altered and the behavious could be a metabolic phenotypic marker for the T cell before his coming at skin lesions.

Adult↗

[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↗

Mycosis fungoides with pulmonary involvement. Cytopathologic findings.

In order to define the cytologic features of pulmonary involvement by mycosis fungoides, 15 respiratory cytology specimens from four patients with biopsy-proven pulmonary mycosis fungoides were reviewed. The presence in sputum smears of occasional small or large cerebriform mononucleated cells against a background of numerous atypical lymphocytic cells permitted an antemortem cytologic diagnosis of probable or definite dissemination of mycosis fungoides with pulmonary involvement. Similar cells were seen in aspiration smears. The lymphocytic infiltrates were similar to those in corresponding skin biopsies in each case. The distinctive cytologic findings in these cases may therefore help to determine the underlying etiology of pulmonary lesions and may contribute to the antemortem diagnosis of visceral dissemination of mycosis fungoides.

Biopsy, Needle↗

Prognostic factors in erythrodermic mycosis fungoides and the Sézary syndrome.

BACKGROUND AND DESIGN: There are no large studies evaluating patients with erythrodermic mycosis fungoides and Sézary syndrome to determine the important prognostic factors that may influence survival. This is important since new treatment modalities have been proposed as superior to existing primary therapies. We performed a retrospective cohort study of 106 patients with erythrodermic mycosis fungoides and Sézary syndrome, followed up in the Stanford (Calif) Mycosis Fungoides Clinic, to define the important prognostic factors in this group. RESULTS: Patients younger than 65 years have a more favorable survival profile than those 65 years or older (P < .005). Longer duration of symptoms before diagnosis ( > or = 10 years) tends to be associated with more favorable prognosis (p = .055). Lymph node stage is significantly correlated with survival; patients with overall stage III disease have more favorable prognosis than those with stage IV disease (P < .001). Patients with circulating Sézary cells in their blood have a significantly worse prognosis than those without (P < .005). Patient sex or race had no significant effect on overall survival outcome. Three distinct prognostic groups were identified, "favorable," "intermediate," and "unfavorable," according to the number of unfavorable prognostic factors (P < .005). The median survival in each group is 10.2, 3.7, and 1.5 years, respectively. CONCLUSIONS: In patients with erythrodermic mycosis fungoides and Sézary syndrome, the important prognostic factors are patient age at presentation, the overall stage, and peripheral blood involvement. Survival varies widely, depending on these variables. These prognostic factors should be evaluated when analyzing survival and/or treatment efficacy data of these patients.

Adult↗

Detection of clonal T-cell receptor gamma gene rearrangements with the use of the polymerase chain reaction in cutaneous lesions of mycosis fungoides and Sézary syndrome.

BACKGROUND AND DESIGN: We used the amplification of junctional V (variable)-J joining sequences of the rearranged T-cell receptor gamma (TCR gamma) genes by polymerase chain reaction for rapid and sensitive detection of a clonal T-cell population in a total of 51 skin specimens obtained from 45 patients with mycosis fungoides, five patients with Sézary syndrome, and 29 patients with chronic inflammatory dermatoses. RESULTS: A clonal TCR gamma gene rearrangement was present in all tumors (3/3, 100%) and in most infiltrated plaques (16/22, 73%) and erythrodermas (10/12, 83%). In the patch stage, a clonal subset was found in more than half of the cases (8/14, 57%), whereas no clonality was observed in the controls. We also amplified the V-J sequences of the Igh locus coding for the heavy chain of immunoglobulins, without evidence of clonal rearrangement. These data were compared with those from in situ immunophenotypic analysis. Moreover, by using the same assay with successive dilutions of standard clonal T-cell DNA, a semiquantitative study of the T-cell clone was carried out in some cases. The highest ratios of clonal DNA were observed in advanced stages. CONCLUSIONS: These data validate polymerase chain reaction V gamma-J gamma as a rapid, sensitive tool that can be used in the routine analysis of clonality in cutaneous lesions of mycosis fungoides and in the early diagnosis of mycosis fungoides and Sézary syndrome. Semiquantitative studies suggest that the malignant T-cell clone follows a selective process during the course of the progressive form of mycosis fungoides.

Clone Cells↗

Treatment of advanced mycosis fungoides and Sézary syndrome with continuous infusions of methotrexate followed by fluorouracil and leucovorin rescue.

BACKGROUND AND DESIGN: The treatment of advanced mycosis fungoides is a therapeutic challenge. A variety of treatment approaches have been used. In our experience, chemotherapy has been most useful. The purpose of this study was to evaluate the effectiveness of the synergy previously demonstrated between methotrexate and fluorouracil in the treatment of advanced mycosis fungoides. Ten patients with mycosis fungoides and Sézary syndrome stages IIa (n = 1), II-b (n = 4), III (n = 1), IVa (n = 2), and IVb (n = 2) were treated with sequential methotrexate followed by fluorouracil and leucovorin rescue. Each patient received several courses of chemotherapy at varying intervals, as required for control of their disease. RESULTS: The duration of treatment ranged from 3 to 78 months, with an average duration of 33 months. The number of cycles of chemotherapy administered to each patient ranged from five to 45, with an average of 18 infusions per patient. The average survival in patients with tumors was 5.25 years, with a median survival of 6 years. Eight of 10 patients achieved at least 80% clearing and the remaining two achieved at least 60% clearing. Adverse reactions were minimal and included nausea and vomiting, mucositis, and leukopenia in only one patient. CONCLUSION: Sequential methotrexate and fluorouracil chemotherapy is an effective and safe treatment for advanced mycosis fungoides and Sézary syndrome. This regimen is extremely well tolerated, with minimal toxic side effects.

Adult↗

Follicular mycosis fungoides. A clinical and histologic variant of cutaneous T-cell lymphoma: report of two cases.

We report two cases of mycosis fungoides with marked, pleomorphic follicular manifestations. Follicular hyperkeratosis, comedo-like lesions, acquired epidermal cysts, and patchy alopecia developed in various locations in both patients. Findings of histopathologic and immunohistochemical studies showed atypical CD4+ T lymphocytes infiltrating the follicles without follicular mucinosis. Focal expression of intercellular adhesion molecule type 1 was observed within the cyst walls. These findings suggest that the follicular lesions were specific for mycosis fungoides. These manifestations represent a distinct clinical and histologic form of mycosis fungoides. This variant probably accounts for cases of mycosis fungoides with clinically suspected alopecia mucinosa in which follicular mucinosis cannot be histologically proved.

Aged↗

[Mycosis fungoides in a Gabonese patient infected with HTLV-I].

Association of human T-lymphotropic virus type-1 (HTLV-1) with T-cell malignancy is well-known but its relationship with mycosis fungoides is controversial. Typical mycosis fungoides was diagnosed at tumor stage in a 58-year-old Gabonese woman also infected with HTLV-1. Infection with lymphoma of the skin is uncommon in Africa but it is probably underestimated. Association of mycosis fungoides with retrovirus infection could be coincidental since there is a high prevalence of HTLV-1 in Gabon and the only currently recognized association is T-cell leukemia/lymphoma. However recent data indicate the presence of similar retrovirus particles and a common tax gene in the monocytes of most patients presenting mycosis fungoides.

Fatal Outcome↗

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↗

[Efficacy of combination chemotherapy with miconazole and G-CSF in deep mycosis accompanying hematological diseases].

In order to examine the efficacy of the combination chemotherapy with miconazole and G-CSF, patients with deep mycosis and suspected deep mycosis were divided into 3 groups. Group I: miconazole and G-CSF were administered simultaneously. Group II: miconazole was administered later during G-CSF administration. Group III: only miconazole was administered. Of a total of 117 cases 105 cases were analyzed including group I 37 cases, group II 39 cases and group III 29 cases, excluding 12 dropout and inadequate cases. Of the 105 cases, deep mycosis were 31 and suspected deep mycosis were 74, and underlying diseases were hematological malignancies such as leukemias. Efficacy judged mainly by the change of fever was 62.2% (23/37) in group I, 43.6% (17/39) in group II, and 41.4% (12/29) in group III, respectively. Efficacy was better in the patients whose neutrophil counts increased from less than 500/microliters to more than 500/microliters (group I 75.0%, group II 72.7%) than in the patients whose neutrophil counts were less than 500/microliters throughout the time of miconazole administration (group I 33.3%, group II 33.3%). Adverse effects were minimal in 3 groups (group I 15.4%, group II 17.4%, group III 15.6%). It is concluded that the combination with miconazole and G-CSF is effective in the treatment of deep fungal infections.

Adult↗