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PubMed1983

OKT 9 reactivity in mycosis fungoides and large plaque (atrophic) parapsoriasis.

A monoclonal antibody OKT 9 which detects a determinant expressed by a variety of proliferating cell types has been recently developed. This antibody was used in conjunction with the immunoperoxidase technique to study the cutaneous lymphoid infiltrates of nine patients with mycosis fungoides, one patient with lymphomatoid papulosis, two patients with Sézary syndrome, and ten patients with large plaque atrophic parapsoriasis (a condition which may terminate in overt mycosis fungoides.) OKT 9 reactive cells were identified in all cases of mycosis fungoides examined, in one case of lymphomatoid papulosis, one of two cases of Sézary syndrome, and one of ten cases of large plaque atrophic parapsoriasis. These results suggest that further studies using OKT 9 should be performed to assess whether OKT 9 reactivity may be used as a prognostic marker in cutaneous lymphomas and prelymphomas.

Antibodies, Monoclonal↗
PubMed2004

An open trial of climatotherapy at the Dead Sea for patch-stage mycosis fungoides.

BACKGROUND: Climatotherapy at the Dead Sea (CDS) is a well-established therapeutic modality for moderate to severe psoriasis vulgaris, resulting in sustained remissions. It has also been found to be effective for atopic dermatitis, another T-cell-mediated dermatosis. OBJECTIVE: We sought to prospectively evaluate the efficacy of CDS in patch-stage mycosis fungoides. METHODS: A total of 12 patients with patch-stage mycosis fungoides (6 with stage IA and 6 with stage IB) were treated with CDS as monotherapy for 28 consecutive days according to the protocol for psoriasis, ie, a gradual increase of sun exposure to a maximum of 3 hours daily. RESULTS: A total of 9 patients achieved a complete clinical response (CCR), defined as no disease activity present; 2 achieved an almost CCR, defined as the reduction by more than 90% of disease activity; and 1 achieved a partial response, ie, reduction by more than 50% of disease activity. A CCR was achieved in all the patients with stage IA disease and in 3 of the 6 patients with stage IB disease. Of the 9 with a CCR, 6 also showed histopathologic clearing. Duration of the remissions, during which no therapy was allowed except for emollients, lasted from 2 to 9 months (mean: 5 months). No serious short-term side effects were recorded. CONCLUSION: CDS appears to be an effective, well-tolerated therapy for patch-stage mycosis fungoides.

Adolescent↗
PubMed1975

Mycosis fungoides with marked brain involvement--a case report.

The clinical course of a patient with mycosis fungoides, which could be confirmed histologically in the premycotic stage, 2 years after the onset of the disease is reported. The disease led to death after a further 3 years, during which the infiltrative and tumorous stage had appeared. At autopsy, apart from the skin changes typical for mycosis fungoides, involvement of the pia mater, plexus chorioideus, cerebrum and cerebellum was established. This constituted the only manifestation of the disease in the internal organ systems. There were no detectable neurological defects.

Adolescent↗
PubMed1989

Unilesional mycosis fungoides: clinical, microscopic and immunophenotypic features.

Seven cases are reviewed in which the histologic and immunohistologic features were those of mycosis fungoides, although the patients presented with solitary lesions which did not recur after local therapy (excision in six, radiotherapy in one), with follow-up of 10 months to 18 years. Immunophenotypic staining of paraffin-embedded tissue revealed a predominant T-lymphocyte proliferation in all cases, None of the four cases studied for BER-H2(Ki-1) were positive, in contrast to the "activated" types of spontaneously regressing lymphoid lesion, lymphomatoid papulosis and "Ki-1" lymphoma. Electron microscopy in one case demonstrated the highly convoluted nuclei of the T cells within the infiltrate. These seven cases represent examples of unilesional mycosis fungoides in which the disease has not recurred after therapy, despite features indistinguishable from those of typical generalised mycosis fungoides-type of cutaneous T-cell lymphoma.

Adult↗
PubMed1985

Conjunctival involvement with mycosis fungoides in a patient receiving PUVA therapy.

A 40-year-old woman had documented mycosis fungoides of the skin. Over a period of five years, she had undergone intermittent treatment with psoralen long-wave ultraviolet light (PUVA) therapy. Despite regression of the skin lesions, tumor plaques developed in her eyelid and conjunctiva within the area shielded by dark glasses. Light and electron microscopic examination of a biopsy from the conjunctival plaque confirmed that the lesion was a deposit of mycosis fungoides. Local radiotherapy resulted in complete regression of these ocular lesions. The conjunctiva may well be an iatrogenic "sanctuary site" when this disease is treated with PUVA therapy.

Adult↗
PubMed1979

[Manifestation of a malignant lymphoma of high grade malignancy in the terminal stage of mycosis fungoides. Enzyme-cytochemical and immunocytological investigations (author's transl)].

Report on a patient with mycosis fungoides, in whom after a long course the disease developed rapidly into a malignant lymphoma of high grade malignancy. The diagnosis was confirmed by cytomorphological and enzyme-cytochemical methods (demonstration of hydrolytic enzymes in cryostat sections, cutaneous smears and cell suspensions extracted from skin lesions), as well as by immunocytological methods (differentiation of lymphocyte sub-populations). In the tumour stage of mycosis fungoides, a polymorphous infiltrate composed of lymphocytoid and monocytoid cells prevailed, while a proliferation of lymphoblastic cells was present in the terminal stage of the disease. These cells showed no longer the properties of mature lymphocytes and differed also in their enzyme-cytochemical pattern.

Histocytochemistry↗
PubMed1979

DNA-cytophotometry of lymph node imprints from patients with mycosis fungoides.

To obtain objective criteria for early diagnosis of lymph node involvement in patients with mycosis fundoides, Feulgen DNA-cytophotometry was carried out in lymph node imprints from patients with mycosis fungoides. The lymph nodes of 3 patients with lymph nodes, showing partial or total replacement by atypical lymphoreticular tissue histologically, showed a polyploid and aneuploid DNA distribution. Eleven out of 22 patients with dermatopathic lymphadenopathy both with or without early involvement histologically had an abnormal DNA histogram with hypertetraploid DNA values. Four of these 11 died, 5 had a partial remission in response to therapy and 2 had sustained remission during the follow-up period of 5 yr. The other 11 patients had a normal DNA distribution. Of these 11, one died and 10 achieved sustained remission after therapy. There is a good correlation between the DNA-cytophotometric results and histology of the lymph nodes. On the basis of these results DNA-cytophotometry may be considered an additional and objective aid in the diagnosis of lymph node involvement in mycosis fungoides.

Adult↗
PubMed2003

Phase 1/2 pilot study of methotrexate-laurocapram topical gel for the treatment of patients with early-stage mycosis fungoides.

OBJECTIVES: To assess the safety and tolerability of a topical gel formulation combining methotrexate and laurocapram and to obtain preliminary information on the therapeutic potential of methotrexate-laurocapram in patients with early-stage mycosis fungoides (stage IA or IB). DESIGN: An open-label, phase 1/2 pilot study. SETTING: Two academic referral centers. PATIENTS: Ten patients 18 years or older with histologically confirmed stage IA or IB mycosis fungoides. Intervention The gel formulation of methotrexate-laurocapram was applied to the total body surface, excluding genital, perianal areas, nipples, face, and skin under the breasts, on an every-other-day basis for 24 consecutive weeks. MAIN OUTCOME MEASURES: The safety of methotrexate-laurocapram was assessed in this study by reviewing adverse events and laboratory data. Efficacy outcomes included changes in lesion condition and severity assessments, reduction in area of sample lesions, and the investigator's global evaluation. RESULTS: Adverse events consisted of skin reactions of mild severity. No clinically significant laboratory abnormalities were observed. Based on the investigator's global evaluation at the end of the treatment phase (week 24), 7 (78%) of 9 patients demonstrated a slight-to-moderate response to treatment with methotrexate-laurocapram. Statistical significance (P =.049) was reached for induration and pruritus, a trend (P =.10) was observed for erythema, and no change was found for scaling (P =.37). CONCLUSIONS: These findings indicate that the topical administration of methotrexate-laurocapram is safe and in general well tolerated. This treatment may represent a new therapeutic potential for patients with mycosis fungoides.

Administration, Topical↗
PubMed1983

Demonstration of a subpopulation of Ia+ T-helper cells in mycosis fungoides and the Sézary syndrome.

This is a report of the finding of a T-cell subpopulation bearing T-helper cells and Ia antigens in specimens of skin from patients with mycosis fungoides and the Sézary syndrome. Frozen sections of skin tissue from eight patients examined with monoclonal antibodies against mature T-cells, helper T-cells, suppressor T-cells, and Ia antigens exhibited similar staining patterns by a modified immunoperoxidase method. Antibodies against mature T-cells and helper T-cells stained 70-80% of the lymphocytes in the dermis. The antibody defining the phenotype of suppressor T-cells labelled 5-10% of the lymphocytes scattered throughout the lesions. Eighty to 90% of the lymphocytes took the stain for Ia antigen. Anti-thymocyte antibody (OKT6) stained cells in both the epidermis and dermis of the specimens. Of nonmalignant conditions examined, lesions from five cases of lichen planus exhibited a quantitatively different staining pattern than that of mycosis fungoides in that the number of T-helper cells was about equal to the number of T-suppressor cells. The findings reported are evidence for a homogeneous population of T-helper, Ia-positive lymphocytes in the cutaneous lesions of mycosis fungoides and the Sézary syndrome.

Adult↗
PubMed1981

Mycosis fungoides in the maxillary sinus and oral cavity: report of two cases.

Two cases of biopsy-proven mycosis fungoides are described. Case 1 appears to be the first reported instance of mycosis fungoides in the maxillary sinus. Case 2 involves persistently recurring lesions within the oral cavity, which appear to have been placed in a long-term remission by a full course of cobalt irradiation therapy to the entire oral cavity.

Aged↗
PubMed1990

Mycosis fungoides in children and adolescents.

The clinical and histologic findings in five young patients with mycosis fungoides are reviewed. The skin specimen from a 16-year-old boy had an infiltrate predominantly Leu-1+, Leu-4+, Leu-3a + 3b+, OKT6+, Leu-2a-, Leu-8-, and Leu-9-, and rearrangements were noted in T cell receptor gene (beta chain [constant region probe] and gamma chain [J region probe]); no rearrangements were found in a histologically normal lymph node or peripheral blood. The skin specimen from an 11-year-old girl contained predominantly Leu-4+ and Leu-9+ lymphoid cells. No T cell receptor gene rearrangements were found in the skin or in an involved lymph node. The variations in clinical, histologic, and immunopathologic features suggest that mycosis fungoides in young patients represents a heterogeneous subgroup of cutaneous T cell lymphoma.

Adolescent↗
PubMed1987

Granulomatous reactions in mycosis fungoides.

Granulomatous reaction characterized by the formation of noncaseating accumulations of epithelioid histiocytes and multinucleated giant cells of the foreign-body type is a rare, poorly understood, and generally ignored phenomenon seen in various types of lymphoma. Its presence in cutaneous infiltrates of mycosis fungoides is equally unusual, but some favorable prognostic significance has been ascribed to it previously. In this paper, four patients with typical mycosis fungoides and granulomas demonstrated histologically in their cutaneous infiltrates are presented. All four died of disseminated disease with evidence of central nervous system involvement in three of them. These clinical histories lend no support to the notion that granulomatous mycosis fungoides is a benign variant of this lymphoma. Relevant literature is reviewed and discussed.

Aged↗
PubMed1992

Combined treatment with oral etretinate and electron beam therapy in patients with cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome).

BACKGROUND: Total skin irradiation for early-stage mycosis fungoides produces clinical remission in 90% of patients, but the median duration of remission is only 2 to 4 years. Etretinate has proven efficacy in advanced mycosis fungoides. Its potential as adjuvant therapy to radiation could be limited by toxicity from the combination. OBJECTIVE: Our purpose was to determine whether oral etretinate at 1 mg/kg can be combined with 35 Gy of radiation without intensifying or prolonging the radiation reaction and to determine the relapse-free survival rate. METHODS: Twenty-three patients began etretinate on day one of radiation and the dose was reduced for nose bleeds, dry skin, or hyperlipidemia. During the reaction skin toxicity questionnaires were completed weekly. Nine concurrent patients receiving only radiation completed identical forms. RESULTS: Etretinate did not intensify the skin reaction but did prolong it by 2 weeks. At a median follow-up of 2 years the relapse-free survival rate in complete responders was similar to stage-matched concurrent and historic control subjects. CONCLUSIONS: Both radiation and etretinate can be given together with acceptable toxicity and without compromising either therapy.

Administration, Oral↗
PubMed1978

Kaposi's varicelliform eruption in mycosis fungoides.

Kaposi's varicelliform eruption, caused by herpes simplex type 1, developed in a patient with mycosis fungoides four days after initiation of photochemotherapy with methoxsalen plus long-wave ultraviolet light (PUVA). He had a history of recurrent localized herpes infection. Vidarabine (adenine arabinoside) was used as treatment. Rechallenge with PUVA did not provoke another herpes infection. Kaposi's varicelliform eruption is rare in mycosis fungoides. Vidarabine may prove to be useful in the treatment of widespread herpes simplex.

Humans↗
PubMed1994

Topical carmustine (BCNU) for patch/plaque mycosis fungoides.

This article summarizes experience with topical carmustine (BCNU) solution in 172 patients with patch/plaque stage mycosis fungoides. At 36 months 92% of stage-T1 (less than 10% skin involvement) and 64% of stage-T2 (10% or more skin involvement) patients were either in complete or partial remission. The most frequent side effect was an erythematous reaction which was followed by a benign-type telangiectasia in some patients. No secondary skin cancers were seen. Bone marrow depression was uncommon. Topical BCNU is an effective treatment for patch/plaque stage mycosis fungoides.

Administration, Topical↗
PubMed2007

Immunohistochemical detection of protein gene product 9.5 (PGP 9.5) in canine epitheliotropic T-cell lymphoma (mycosis fungoides).

Protein gene product 9.5 (PGP 9.5), a ubiquitin COOH-terminal hydrolase initially considered specific for neural and neuroendocrine tissues, is expressed in a variety of epithelial and mesenchymal tumors. During immunohistochemical evaluation of a cutaneous epitheliotropic T-cell lymphoma (mycosis fungoides [MF]) in a dog, strong reactivity for PGP 9.5 was observed. This unexpected result prompted us to examine PGP 9.5 immunoreactivity in 13 additional cases of canine mycosis fungoides. All tumors were confirmed as T-cell epitheliotropic lymphoma by histopathology and immunohistochemistry for CD3. Eight of 14 cases were positive for PGP 9.5, with reactivity mainly in the cytoplasm and less commonly in the nucleus. One case had strong reactivity in the cell membrane, sometimes with concurrent paranuclear staining. Immunoreactivity did not correlate with location (epidermal, dermal, and adnexal) of tumor cells. Disease outcome did not vary between PGP 9.5-positive and negative tumors. Although PGP 9.5 immunoreactivity in MF did not predict tumor behavior in these dogs, it has had prognostic value in certain human carcinomas. This unexpected staining of lymphocytes in mycosis fungoides with an antibody to PGP 9.5 demonstrates its presence in nonneuroendocrine tumors and precludes its use as the sole diagnostic marker in discrete cell tumors in the skin.

Animals↗
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↗