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PubMed1993

Intramuscular low dose alpha-2B interferon and etretinate for treatment of mycosis fungoides.

BACKGROUND: Mycosis fungoides is a lymphoma of cutaneous origin characterized by a proliferation of cells with a T phenotype. METHODS: In this pilot study, 13 men with mycosis fungoides in various stages were treated with alpha-2b interferon and etretinate. RESULTS: In ten of them, such a therapy proved to be effective (7 complete responses, 3 partial responses), sometimes with prolonged remissions (up to 20 months, and still persistent) after suspension of the drugs. CONCLUSIONS: We chose low-dose interferon administration in order to prevent side effects, which are said to be dose-dependent. In our experience this is not true, but this drawback seems to be overcome by the very good, sometimes spectacular, response to this combination therapy, particularly in low stage forms of the disease. This fact, compared with results provided by other groups, prompts us to plan new research protocols based on associations of retinoids with different interferon types (or even associations of different interferons), because we believe they will have an important place in treatment of cutaneous T-cell lymphoma in future.

Adult↗
PubMed1997

Mycosis fungoides: an Australian experience.

Analysis was undertaken of 107 patients with mycosis fungoides who presented to St. Vincent's Hospital, Melbourne, during 1977-95. The mean age at diagnosis (55 years) was significantly older than the mean age of onset of symptoms (48 years). The mean duration of follow-up was 7.7 years. Urban residence was over-represented in this group of patients and in those with mycosis fungoides recorded at the State Cancer Registry. Eighty-four per cent were diagnosed at Stage I or II of the disease. Presentation with disease more advanced than Stage I was more likely to be found in males. Disease localized only to the trunk accounted for 45% of all patients. The mean number of biopsies prior to diagnosis was 1.4 but two-thirds were diagnosed at first biopsy. Almost 85% of patients had as their initial treatment either PUVA (44.9%), topical steroids (20.6%) or topical nitrogen mustard (18.7%) and 57% received only one or two treatment modalities during the period of the study. Stage at presentation was not related to the likelihood of clearance following treatment, recurrence, progression to a more advanced stage of disease or survival.

Adolescent↗
PubMed2004

Clinicopathological spectrum of mycosis fungoides.

Cutaneous lymphomas represent a heterogeneous group of T-, NK- and B-cell neoplasms, with mycosis fungoides (MF) being the most common subtype. MF has a plethora of clinicopathological manifestations. Many variants of this lymphoma differ substantially from the 'classical' Alibert-Bazin disease and are therefore sometimes referred to as 'atypical' forms of the disease. This review addresses the whole clinicopathological spectrum of mycosis fungoides with respect to epidemiology, clinical, histopathological, immunophenotypic and genotypic features and the clinical course and prognosis of its variants: classical, erythrodermic, follicular, syringotropic, bullous/vesicular, granulomatous, poikilodermic, hypo- and hyperpigmented, unilesional, palmoplantar, hyperkeratotic/verrucous, vegetating/papillomatous, ichthyosiform, pigmented purpura-like, pustular and mucosal involvement in MF.

Humans↗
PubMed1980

Mycosis fungoides: retrospect, current understanding, and prospect.

After a brief historical view of mycosis fungoides from Alibert's time to the present, the authors go on to detail the average biologic course of mycosis fungoides, give a protocol for investigating patients with disease as extensively as modern means permit, review past and present staging schemes, and finally, review treatment as practiced today and speculate upon what may yet be practiced to the better in the future.

Humans↗
PubMedDate not supplied

Hypopigmented mycosis fungoides associated with atopy in two children.

Mycosis fungoides is very rare in children. Hypopigmented lesions of this disease are usually observed in dark-skinned individuals and often show a T supressor CD8(+) phenotype. Two Caucasian children with predominantly hypopigmented lesions of mycosis fungoides are presented here. Atopy was a concomitant feature in both.

Child, Preschool↗
PubMed1979

Target role of Langerhans cells in mycosis fungoides: transmission and immuno-electron microscopic studies.

Langerhans cells of the epidermis are "special" macrophages, as indicated by their expression of Fc and C3 receptors and Ia antigen. Functionally, they can replace macrophages in presentation of antigens to T cells and in the MLR. Ultrastructural and immunologic studies were carried out on epidermal samples of mycosis fungoides. As in contact allergy, apposition of lymphocytes to Langerhans cells was noted. Destruction of Langerhans cells appeared to act as the focus for the development of Pautrier microabscesses. This destruction was accompanied by ingress of histocytic cells, probably related to the stem cell population for Langerhans cells. The target cell role of Langerhans cells appears to be similar in contact allergy and mycosis fungoides.

Antigens↗
PubMedDate not supplied

Early diagnosis of Mycosis fungoides: vacuolar interface dermatitis.

The earliest detectable histologic alteration in patients developing mycosis fungoides is vacuolar interface dermatitis. Effacement of the epidermis is common. This is followed sequentially by invasion of the epidermis by mononuclear cells both singly and in groups, epidermal hyperplasia and finally the development of easily recognized Pautrier microabscesses as well as of characteristic atypical "mycosis" cells.

Dermatitis↗
PubMed1990

Carbamazepine-induced eruption histologically mimicking mycosis fungoides.

Carbamazepine is an important drug used in the management of seizures, trigeminal neuralgia, and chronic pain syndromes. It has been associated with a variety of adverse skin reactions including urticaria, lichenoid eruptions, erythroderma, erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. A 39-year-old white male had been started on carbamazepine for intractable pain which resulted from a right foot crush injury. Approximately 3 months after the start of therapy, the patient had developed a generalized skin eruption following an entire day of sun exposure. Skin biopsies revealed an atypical lymphoid infiltrate in the dermis with collections of the atypical lymphocytes within spongiotic vesicles in the epidermis, suggestive of mycosis fungoides. The patient was treated with systemic prednisone. Subsequent biopsies failed to reveal atypical lymphocytes. Previous reports have described spongiotic eruptions with foci of atypical lymphocytes in contact dermatitis and in patients treated with phenytoin. To the best of our knowledge, this is the first reported case of a carbamazepine-induced eruption simulating mycosis fungoides histologically.

Adult↗
PubMed1991

Application of DNA flow cytometry from paraffin-embedded tissue to the diagnosis of mycosis fungoides.

The distinction of mycosis fungoides from benign inflammatory lesions is sometimes difficult by conventional histological techniques. Aneuploidy, a feature often associated with malignant tumors, can be assessed even in tissue routinely processed in paraffin using the flow cytometric technique of Hedley and associates. In many tumor systems, there are significant diploid clones. We have evaluated the flow cytometric DNA ploidy of paraffin-embedded tissue in the diagnosis and prognosis of mycosis fungoides (MF). We studied 22 cases of MF and 10 control cases of inflammatory skin lesions with epidermal involvement. Aneuploidy was found in 27% of the MF cases, but in none of the controls (ED). Aneuploid features were seen in 23% of tissues from early stage disease. Aneuploidy did not correlate with atypia, epidermotropism, or number of mitoses. There was a trend towards showing adverse outcome in those patients with aneuploid lesions. The detection of aneuploidy might be helpful for early diagnosis of MF.

Aneuploidy↗
PubMed1994

Mycosis fungoides skin lesions contain CD8+ tumor-infiltrating lymphocytes expressing an activated, MHC-restricted cytotoxic T-lymphocyte phenotype.

In prior studies, we showed that most CD8+ cells infiltrating skin lesions of CD3+CD4+ mycosis fungoides were CD3+ T-lineage tumor-infiltrating lymphocytes (TIL) whose overall phenotype was suggestive of MHC-restricted cytotoxic T lymphocytes (CTL). However, their lack of cytotoxic-associated granzyme A mRNA suggested that they might be unactivated CTL precursors. In this study, we used single- and double-label immunohistologic techniques to assess the expression of TIA-1-reactive protein and HLA-DR by these CD8+TIL. Monoclonal antibody TIA-1 recognizes a novel family of proteins expressed preferentially by cytotoxic cells, including some that lack granzyme A. HLA-DR is a marker of T-cell activation. Single-label studies of 32 cases showed that CD8+TIL and TIA-1+ cells constituted a variable minority of the total cellular infiltrate and had a similar distribution. Double-label studies of 14 cases showed that in most instances the aggregate phenotype of the majority of CD8+TIL was CD3+TIA-1+HLA-DR+CD56-CD57-. These findings suggest that many of the CD8+TIL within skin lesions of CD3+CD4+ mycosis fungoides are activated, MHC-restricted CTL.

Antibodies, Monoclonal↗
PubMed2005

Narrow band UVB (311 nm), psoralen UVB (311 nm) and PUVA therapy in the treatment of early-stage mycosis fungoides: a right-left comparative study.

BACKGROUND: Psoralen ultraviolet A (PUVA) is a widely used first-line therapy for treatment of early cutaneous T-cell lymphoma. Narrow band UVB (UVB-NB) (311 nm) has been recently introduced as another effective line of treatment. It is postulated that the efficacy of UVB-NB could be enhanced by addition of psoralen. AIM: The aim of the present work was to compare the clinical and histopathologic efficacy of PUVA and UVB-NB in the treatment of early-stage MF (stages IA, IB and IIA), and to evaluate whether psoralen adds to the efficacy of UVB-NB or not. PATIENTS AND METHODS: Twenty patients (stage IA, IB or IIA) were divided into two equal groups: group I received UVB-NB on the right body half vs. PUVA on the left side of the body for 48 sessions, and group II received PUVB-NB on the right side of the body vs. PUVA on the left side for 36 sessions. The sessions were administered three times weekly. RESULTS: In group I, almost equal results were obtained on both sides, i.e., UVB-NB and PUVA were equally effective in the treatment of early stages of MF, both clinically and histopathologically. In group II, PUVB-NB was found to be as effective as conventional PUVA in the treatment of early-stage mycosis fungoides, also on both clinical and histopathological grounds. CONCLUSION: UVB-NB phototherapy should be included among the initial therapeutic options of mycosis fungoides in view of its efficacy, convenience, and likelihood of fewer long-term adverse effects. Addition of psoralen does not seem to enhance its therapeutic efficacy.

Adult↗
PubMed2005

Vesicular mycosis fungoides.

A 62 years old patient presented with rapidly progressive mycosis fungoides. Lesions of the face, head, trunk and limbs exhibited numerous vesicles and erosions. Histopathology showed marked spongiosis and intraepidermal blisters, as well as invasion of the epidermis by atypical lymphocytes, which was confirmed by immunohistochemistry. The vesicular variant of mycosis fungoides is rare and associated with a poor prognosis.

Back↗
PubMed2001

Autologous peripheral blood stem cell transplantation in tumor-stage mycosis fungoides: predictors of disease-free survival.

Nine patients with mycosis fungoides (age range 27-67) underwent autologous peripheral blood stem cell transplantation (PBSCT). All patients had tumor-stage disease, and four had lymph node involvement. Eight patients exhibited a peripheral blood T cell clone using PCR/SSCP analysis of the TCR gamma gene, six prior to harvest and two at the time of harvest. Mobilization of CD34+ stem cells was achieved with etoposide and G-CSF. Harvested cells were positively selected for CD34. After negative selection for CD4 and CD8, only two samples became PCR negative. Conditioning prior to reinfusion of stem cells was achieved with various combinations of total skin electron beam (TSEB), total body irradiation (TBI), and chemotherapy, depending upon the patient's prior exposure to radiotherapy. One patient failed to engraft and died of candidal septicemia 15 days posttransplant. The other eight patients achieved complete remission, but this was short-lived in four (median disease-free survival [DFS] = 2 months) and prolonged in three (median DFS 11 months). Those with a short DFS were distinguished by rapid tumor onset prior to transplant but not by stage at transplant. Loss of a detectable T cell clone after manipulation of the harvest did not discriminate between the two groups, but rapid relapsers had been subjected to a greater degree of T cell depletion, possibly indicating a compromised cytotoxic response post-PBSCT. The median survival of the cohort is four years from tumor onset, 15 months from PBSCT, and 27 months from the date a peripheral blood clone was first detected in the presence of tumor-stage disease. Rapid relapse was associated with poor overall survival. Our data demonstrate the value of PBSCT for inducing remission in tumor-stage mycosis fungoides. Reinfusion of neoplastic cells could be avoided by harvesting stem cells at an earlier stage in the disease process, preferably before a T cell clone is detectable in the peripheral blood. Alternatively T cell depletion should be restricted to the CD4 subset.

Adult↗
PubMed1991

Intraocular involvement with subretinal pigment epithelium infiltrates by mycosis fungoides.

We report a case of intraocular mycosis fungoides in a 48-year-old man. The patient presented with decreased visual acuity, white subretinal lesions, and vitritis. Post-mortem histopathology revealed malignant T cell infiltrates consistent with mycosis fungoides in the retina, vitreous, and between the retinal pigment epithelium (RPE) and Bruch's membrane Focal atrophy of the RPE, along with the sub-RPE infiltrates, correlated with the clinically visible fundus lesions.

Bruch Membrane↗
PubMed1977

Photochemotherapy in mycosis fungoides.

Six patients with mycosis fungoides were treated with methoxsalen and long-wave ultraviolet light. They were assessed clinically and histologically before and after treatment. All six patients showed clinical improvement. Histological clearing of both epidermis and dermis occurred in three patients, and epidermal clearing alone occurred in two. Photochemotherapy may have a place in the treatment of the early and intermediate stages of mycosis fungoides, and it may also be useful as an adjunct to other forms of treatment in the more advanced stages.

Adult↗
PubMed1986

Immunocytochemical characterisation of cutaneous lymphomas other than mycosis fungoides.

The immunophenotypic properties of 25 cutaneous non-Hodgkin lymphomas other than mycosis fungoides or Sezary syndrome were investigated and correlated with clinical and histopathological data. The 11 low grade lymphomas were all of B cell origin, whereas the 14 high grade lymphomas comprised B and T cell tumours, true histiocytic proliferations, and one "nul" cell lymphoid neoplasm. For the high grade lymphomas correct prediction of the immunological phenotype based on morphological criteria was only possible in three cases. In contrast, all of the low grade lymphomas showed the non-epidermotropic infiltration pattern considered to be characteristic of cutaneous B cell tumours. For these conditions, however, immunophenotypic investigations provided a convenient means of improving discrimination between benign (polyclonal) and malignant (monoclonal) lesions, and also showed similarities with nodal lymphomas in terms of expression of lymphoid subset markers and composition of the non-neoplastic white cell infiltrate. No differences were identified between primary and secondary or concurrent cutaneous and extracutaneous lymphomas. Cutaneous non-Hodgkin lymphomas other than mycosis fungoides or Sezary syndrome constitute a heterogeneous group of neoplasms and most of these disorders are likely to represent cutaneous equivalents of nodal malignancies. Immunophenotypic investigations form a useful supplement to their histogenetic characterisation and may provide a common conceptual basis for their classification.

Adolescent↗
PubMed1982

Brain biopsy in the diagnosis of cerebral mycosis fungoides.

A case of cerebral mycosis fungoides co-existing with progressive multifocal leucoencephalopathy presented with dementia. Brain biopsy established the diagnosis of mycosis fungoides after cerebrospinal fluid examinations and computerised tomographic scanning of the brain produced non-specific abnormalities.

Biopsy↗
PubMed1989

Peripheral neuropathy associated with mycosis fungoides.

A 56 year old man with acute sensory-motor polyneuropathy associated with mycosis fungoides is described. EMG studies showed diffuse signs of muscle denervation. A skin biopsy specimen showed a lymphocyte infiltration in the dermis, composed of mycosis cells characterised by deep invaginations of the nuclear membrane, and small Pautrier's microabscesses in the epidermis. Sural nerve biopsy revealed endoneurial fibrosis, a decreased number of myelinated fibres and acute axonal degeneration.

Biopsy↗