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PubMed1998

Analysis of T-cell receptor gene rearrangement in lymph nodes of patients with mycosis fungoides. Prognostic implications.

OBJECTIVE: To determine the prognostic value of analyzing lymph node (LN) DNA from patients with mycosis fungoides for the presence of a monoclonal T-cell population. DESIGN: Inception cohort study. SETTING: A tertiary care referral center in Seattle, Wash. PATIENTS: Fifty-five uniformly staged patients with the diagnosis of mycosis fungoides and who had a lymph node biopsy, 21 with clinically abnormal nodes and 34 with normal nodes. MAIN OUTCOME MEASURES: Lymph nodes were evaluated by Southern blot analysis for T-cell receptor beta-chain (TCRB) gene rearrangement and by histopathologic examination for the LN classification using the National Cancer Institute system. Patients were observed clinically for a mean (+/- SD) of 4.7 +/- 3.4 years. RESULTS: Patients with detectable TCRB gene rearrangement in lymph node DNA had an increased likelihood of a poor clinical outcome and a decreased probability of survival (P < .001 for both) compared with patients with the TCRB germline. Although patients with clinically enlarged nodes were more likely to have the TCRB gene rearranged, those with normal nodes and the TCRB gene rearranged also had a poor clinical outcome and a decreased probability of survival. Similar to those with the TCRB gene rearranged, most patients with advanced histopathologic changes (LN3 and LN4) had a poor prognosis. The presence of a rearranged TCRB gene, however, correctly predicted some patients with intermediate LN scores (LN2) who had a poor clinical outcome. CONCLUSIONS: Detection of a monoclonal T-cell population, as demonstrated by a rearranged TCRB gene on Southern blot analysis, in LNs of patients with mycosis fungoides is predictive of a poor clinical outcome and a reduced probability of survival. Lymph node TCRB gene analysis provides additional prognostic information for patients with mycosis fungoides with intermediate LN histopathology.

Antineoplastic Agents↗

PubMed1977

Central nervous system involvement in mycosis fungoides: pre mortem diagnosis by light and electron microsocopic study of the spinal fluid.

Involvement of the brain, spinal cord, peripheral nerves, meninges, and cerebrospinal pathways in mycosis fungoides manifests itself, clinically, as an obscure neurologic disturbance, occurring in patients with cutaneous manifestations of that disorder. Neurologic expressions of this particular cutaneous lymphoma are infrequently reported, in spite of the fact that many patients dying in the course of mycosis fungoides have pathologically demonstrable central nervous system, peripheral nervous system, and meningeal involvement. We report here a patient who incurred devastating neurologic illness as one of the initial features of the course of systemic dissemination of mycosis fungoides. Death occurred nine years after the onset of cutaneous illness. In this instance, cerebrospinal fluid cytologic studies established the diagnosis of nervous system involvement. Mycosis fungoides cells shed into the cerebrospinal fluid were studied by special techniques of light and electron microscopy. Brain scan abnormalities were present, which were clinically correlative with foci of brain involvement. The initial response of patient's nervous system illness to therapy was gratifying but unsustained, and she went on to fatal brain and multiple organ involvement with disseminated mycosis fungoides. The techniques of spinal fluid cytologic diagnosis, confirmatory of the etiology of disease in this case, may have practical appications in the diagnosis of otherwise obscure neurologic disorders.

Adult↗

PubMed1961

Electron beam therapy of mycosis fungoides.

Ionizing radiation in the form of x-ray therapy is the best modality of treatment available at the present time for single, isolated lesions of mycosis fungoides. However, for generalized mycosis fungoides, generalized x-ray therapy is technically difficult and dangerous. It is now possible to employ electron beam therapy for generalized mycosis fungoides, using energies which confine the dose to the superficial layers of the skin and thus avoid hematopoietic injury. A technique for wide field electron beam therapy has been developed for this purpose which has been effective and well tolerated in limited trials to date.

Electrons↗

PubMed1999

Clinical characteristics and long-term outcome of patients with generalized patch and/or plaque (T2) mycosis fungoides.

OBJECTIVES: To study the long-term results of treatment of patients with generalized patch and/or plaque mycosis fungoides and to identify clinical characteristics predictive of survival and response to treatment. DESIGN: A single-center, 35.5-year retrospective cohort analysis. SETTING: Private referral medical center. PATIENTS: One hundred seventy-six patients with generalized patch and/or plaque (T2) mycosis fungoides. MAIN OUTCOME MEASURES: Long-term actuarial survival and freedom-from-relapse results as calculated by the Kaplan-Meier method. RESULTS: The long-term (35.5-year) survival of patients with T2 mycosis fungoides is worse than the expected survival of a race-, age-, and sex-matched control population (P<.001). The median survival of the T2 group is 11.7 years. Patients younger than 58 years (median age) at presentation have a more favorable overall and disease-specific survival than the patients who are 58 years or older (P<.001 vs P<.025). Patient sex or race had no significant effect on overall survival. Patients who presented with palpable clinically significant lymph nodes (stage IIA) had long-term survival results similar to those without lymphadenopathy (stage IB), despite improved freedom-from-relapse outcome for patients with stage IB. Twenty-four percent of patients who progressed to more advanced disease had a lower complete response rate to initial therapy than did other patients (21% vs 65%) (P<.001). Patients who received total skin electron beam therapy had a better complete response rate than patients treated with topical mechlorethamine hydrochloride alone; the relapse-free results were superior in patients with a total dose of 30 Gy or higher and in patients who received topical mechlorethamine as adjuvant therapy following total skin electron beam therapy. Despite differences in freedom-from-relapse results among different treatment groups, long-term overall or disease-specific survivals were not significantly different. CONCLUSIONS: A significant proportion (24%) of patients with generalized patch and/or plaque (T2) mycosis fungoides experience disease progression to a more advanced clinical stage, and nearly 20% eventually die of the disease. Younger patients have a more favorable disease-specific long-term outcome than patients who are older. Presence of lymphadenopathy (stage IIA) at diagnosis does not predict worse long-term survival outcome. Clinical features predictive of disease progression include initial lymphadenopathy (stage IIA) and lack of complete response to initial treatment. Despite superior complete response rate to a 30-Gy or higher dose of total skin electron beam therapy, topical mechlorethamine proves to be a cost-effective initial treatment for patients with T2 disease. The concept of an adjuvant therapy after irradiation is appealing, although it may not lead to improved long-term survival.

Adult↗

PubMedDate not supplied

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↗

PubMed1995

Basaloid folliculolymphoid hyperplasia with alopecia as an expression of mycosis fungoides (CTCL).

Follicular mucinosis, papules, cysts and comedones have been previously described as expressions of follicular involvement by mycosis fungoides. We report a patient with mycosis fungoides who developed extensive alopecia. Multiple scalp biopsies showed perifollicular and intrafollicular infiltrate of lymphocytes but no evident follicular mucinosis. On transverse sections many of the follicles showed an absence of differentiation towards hair sheath, canal, sebaceous gland or hair formation, but instead formed undifferentiated basaloid structures. These basaloid structures showed transition from atrophic telogen follicles to hypertrophic basaloid islands infiltrated by lymphocytes, resembling the pattern previously described in cutaneous lymphadenoma. Immunophenotyping showed a predominance of helper T-cells which, on ultrastructural examination, showed cerebriform nuclei. The unusual histological findings in our case may be analogous to the hyperplasia seen in sweat glands in syringotropic mycosis fungoides (syringolymphoid hyperplasia), and we propose the term basaloid folliculolymphoid hyperplasia to describe this feature. Basaloid follicular hyperplasia has been previously described as a component of follicular mucinosis but may apparently develop in the absence of overt mucinosis.

Aged↗

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↗

PubMed1977

Hodgkin's disease and mycosis fungoides in a married couple.

Hodgkin's disease and mycosis fungoides are malignancies in which there is abnormal T lymphocyte proliferation. The authors report the occurrence of Hodgkin's disease and mycosis fungoides in a husband and wife, respectively. This represents another instance of clustering of cases of T lymphocyte malignancies.

Adult↗

PubMed1984

[Whole-skin electron therapy: a therapeutic concept for mycosis fungoides and Sézary syndrome. 2: Therapy].

The alterations of mycosis fungoides of a malignant T-cell lymphoma are extremely radiosensitive. In the treatment of the cutaneous manifestation of mycosis fungoides, the whole-skin electron therapy is superior to the superficial application of mechlorethamine (NH2), to photochemotherapy with psoralen and ultraviolet radiation as well as to chemotherapy as monotherapy and combined therapy. Complete remissions are to be expected in 93% of all cases after application of a high total dose of more than 30 Gy. After less than 10 Gy, this rate is 18%. A combination of whole-skin electron therapy (30 Gy) with chemotherapy or with a total lymphoid irradiation with 30 Gy represents a new approach for the treatment of advanced tumor stages.

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↗

PubMed2001

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

BACKGROUND: Follicular mycosis fungoides is an unusual variant of mycosis fungoides (MF). Unlike classic MF where atypical lymphocytes show a predilection for the epidermis (epidermotropism), follicular MF displays a malignant lymphocytic infiltrate tropic for hair follicles (folliculotropism). This malignant lymphocytic infiltrate results in follicular disruption typically manifesting clinically as plaques, comedones and follicular papules. METHODS: This report describes a 40-year-old patient with follicular MF presenting as nodules on the face and chest. Histologic examination of the patient's biopsy revealed a folliculocentric infiltrate of atypical lymphocytes with sparing of the epidermis. RESULTS AND CONCLUSIONS: Our case is discussed in the context of previously reported cases of follicular MF; we also include a review of all cases of follicular MF published to date that meet the strict criterion for this diagnosis.

Administration, Topical↗

PubMed1981

[Change of level of mycosis fungoides under systemic PUVA-therapy (author's transl)].

In a patient with mycosis fungoides the typical skin lesions showed a good response to oral photochemotherapy (PUVA), which could be confirmed by serial control biopsies. Twelve months after onset of therapy large subcutaneous nodules appeared, whereas the upper skin remained uninvolved. Two months later the patient died with disseminated visceral involvement, whereas skin lesions were minimal. The case reported here shows that improvement of the skin lesions of mycosis fungoides under systemic PUVA-therapy can lead to misjudgement of the actual course of the disease. PUVA-therapy of mycosis fungoides may cause a change of the involved layers, leading from the cutis to the subcutis, i.e. disappearance of epidermotropic plaque-like infiltrates and appearance of subcutaneous nodules and visceral involvement.

Aged↗

PubMed2005

[Mycosis fungoides with predominant periorificial and mucous involvement].

INTRODUCTION: Specific involvement of the mucous membranes is possible during the course of mycosis fungoides but has seldom been reported, except in postmortem series. A single mucous membrane is most often involved, mainly in the mouth. Such mucous lesions are generally ominous with regard to the general outcome of the disease. OBSERVATION: A 74 year-old woman was investigated for mycosis fungoides complicated with lesions around the mouth and of the mucosa, involving the tongue and esophagus, featuring ulcerated nodules with specific chorion infiltration and epidermotropism. This progression was rapidly followed by a fatal outcome, in spite of various systemic treatments. DISCUSSION: This case report of mycosis fungoides displaying multiple and predominant oral and mucosal involvement of mycosis fungoides is unique. The rapidly unfavorable outcome confirms the ominous prognosis of mucous lesions, whereas no patent visceral extension was detected. The mechanisms underlying the mucous membranes involvement is discussed.

Aged↗

PubMed1977

Mycosis fungoides with rapidly progressive pulmonary infiltration.

A case of mycosis fungoides is reported in which diffuse pulmonary infiltration was noted in six days. Although pulmonary infiltration with the abnormal Sézary cells is a frequent occurrence in the extracutaneous spread of mycosis fungoides, this is the first case report documenting the rapidity of spread.

Aged↗

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↗

PubMed2004

Mycosis fungoides mimicking inflammatory linear verrucous epidermal nevus.

We experienced an unusual case of mycosis fungoides with the clinical and histological features mimicking inflammatory linear verrucous epidermal nevus (ILVEN) in an 11-year-old boy. Localized linear multiple pruritic verrucous confluent papules and plaques appeared on the his left elbow, forearm and hand for 7 months. Skin biopsies showed characteristic findings of mycosis fungoides (e.g. Pautrier's microabscesses, follicular epitheliotropism, wiry bundles of collagen, etc.). T-cell receptor gene rearrangement analysis in the lesional skin demonstrated rearrangement of the gamma chain. RePUVA (systemic PUVA with retinoic acid) therapy improved his skin lesions and pruritus, but these progressed after discontinuation of treatment. Thus, lesions mimicking ILVEN can be an unusual and potentially misleading presentation of mycosis fungoides.

Child↗

PubMed1989

Expression of T-cell receptor antigens in mycosis fungoides and inflammatory skin lesions.

Using immunohistologic methods, we studied the expression of the T-cell receptor (TCR)-associated antigens CD3, TCR-beta, and TCR-delta by cutaneous T cells in mycosis fungoides (MF) (36 patients) and a variety of inflammatory diseases (16 patients). Most T cells in the inflammatory diseases and patch/plaque mycosis fungoides expressed the immunophenotype characteristic of the vast majority of mature peripheral T cells: CD3+ TCR-beta+ TCR-delta-. In contrast, abnormal CD3/TCR-beta antigen expression was seen in 3 of 6 cases (50%) of tumor stage mycosis fungoides. Furthermore, we were able to document its evolution from the normal pattern present in earlier patch/plaque lesions of the two cases in which serial biopsies were available for study. Divergence of epidermal versus dermal CD3/TCR-beta antigen expression was seen in 2 of 34 (6%) of biopsies of patch/plaque mycosis fungoides but not in inflammatory controls. The TCR-delta+ cells were generally rare regardless of diagnosis. We conclude that inflammatory skin diseases and most patch/plaque mycosis fungoides are typically composed of T lymphocytes that resemble mature peripheral T cells in regard to their expression of TCR-associated antigens. In contrast, aberrant patterns of TCR-associated antigen expression can be seen in tumor stage MF, and, more rarely in patch/plaque MF.

Antigens, Differentiation, T-Lymphocyte↗

PubMed1999

Total skin electron radiation for patients with erythrodermic cutaneous T-cell lymphoma (mycosis fungoides and the Sézary syndrome).

BACKGROUND: There is limited published evidence regarding the efficacy of total skin electron beam radiation for patients with the diffuse erythrodermic form of mycosis fungoides. METHODS: Forty-five patients with erythrodermic mycosis fungoides were managed at McMaster University in Hamilton, Ontario, Canada (n=34), and at Yale University (n=11) between 1970 and 1996. All received radiation without neoadjuvant, concomitant, or adjuvant therapies. The median age was 67 years (range, 42-84 years). The male-to-female ratio was 2.2. Fifteen received radiation for the treatment of newly diagnosed disease. There were 28 with Stage III (T4 N0-1 M0), 13 with Stage IVA (T4 N2-3 M0), and 4 with Stage IVB (T4 N0-3 M1) disease, and 21 had blood involvement. The median radiation dose was 32 gray (Gy) (range, 4.8-40 Gy). The median treatment time was 21 days (range, 3-125 days). A technically more intense method of radiation (32-40 Gy and 4-6 MeV electrons) was administered to 23 patients. RESULTS: All patients responded. The rate of complete cutaneous remission was 60%, with 26% remaining progression free at 5 years. Remission was associated with more intense radiation (P=0.014 in multivariate analysis with adjustment for blood and staging information). With the more intense radiation, 74% attained remission, with 36% remaining progression free at 5 years. For 8 patients with Stage III disease without blood involvement, all entered remission, with 69% remaining progression free at 5 years. Twenty of 30 deaths were related to mycosis fungoides. The median overall survival was 3.4 years, with a 10-year estimate of 28%. The median cause specific survival was 5 years, with a 10-year estimate of 43%. Both overall and cause specific survival were associated with an absence of blood involvement (both P<0.03 in multivariate analysis). Age was not a significant factor. Toxicities of radiation were acceptable when radiation was administered over 6-9 weeks at 5 fractions per week. CONCLUSIONS: Total skin radiation is an efficient monotherapy for patients with erythrodermic mycosis fungoides. With more intense radiation, the rate of cutaneous remission is 74%, and 27% remain progression free at 10 years. Radiation may be most efficacious in Stage III, with no blood involvement. When there is blood, lymph node, or visceral involvement, combined modality therapies should be explored.

Adult↗