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Hypopigmented mycosis fungoides: case reports and literature review.

BACKGROUND: Hypopigmented mycosis fungoides is a rare variant of mycosis fungoides (MF) that usually has a predilection for young individuals with dark complexion. OBJECTIVE: The aim is to describe new cases of hypopigmented MF with confirmed T-cell receptor gene rearrangement analysis. METHODS: This article includes case reports and a literature review. RESULTS: Three out of four hypopigmented MF patients had a positive TCR gene rearrangement. A fifth patient is reported who had hypopigmented mycosis fungoides and classical Pautrier microabscesses, for whom no TCR gene rearrangement analysis was performed. CONCLUSION: Although hypopigmented MF has a predilection for dark-complexioned populations, it can also affect Caucasian patients. In challenging cases, polymerase chain reaction can be a useful method for detecting early cases of hypopigmented MF.

Adult↗

Morphometric study of lymphoid cells in mycosis fungoides and its simulators.

The distinction of early stages of mycosis fungoides from benign lymphoid disorders of skin is difficult by conventional histological techniques. We studied 10 cases of mycosis fungoides, 10 cases of large plaque parapsoriasis, 10 cases of other benign lymphoid disorders of skin and 5 cases of lymph nodes. Nuclear area, perimeter of the nucleus, nuclear contour index, cytoplasmic area, form factor and nuclear cytoplasmic ratio as well as DNA-ploidy were determined by image analysis. There were statistically significant difference (P value < 0.05) between all parameters except nuclear cytoplasmic ratio of the lymphoid cells of Mycosis Fungoides and benign lymphoid disorders of skin. Aneuploidy was found in 50% cases of Mycosis Fungoides. Histopathological parameters like epidermotropism pautrier micro-abscess and atypical lymphocytic infiltrate in both epidermis and dermis were more marked in aneuploid than diploid cases. So, the determination of nuclear contour index and DNA-ploidy is of importance to differentiate between Mycosis Fungoides and benign lymphoid disorders of skin.

Adult↗

Photochemotherapy and topical nitrogen mustard in the treatment of mycosis fungoides.

Eight patients with mycosis fungoides limited to the skin have been treated with either topical nitrogen mustard alone or in combination with photochemotherapy. This regime does not prevent contact sensitization to nitrogen mustard but will clear sanctuary sites of disease which may develop when photochemotherapy is used alone.

Administration, Topical↗

Further similarities between Sézary syndrome and mycosis fungoides.

Sézary syndrome and mycosis fungoides, both malignant Non-Hodgkin lymphomas, although characterized as specific entities, show a close relationship. This is based on striking similarities not only uith respect to morphological, cytophotometrical, cytogenetical and immunological findings, but also with respect to a typical common reaction pattern to photochemotherapy.

Aged↗

PUVA treatment in mycosis fungoides.

2 patients with mycosis fungoides at the plaque stage were treated with oral methoxalen and UVA irradiation. 3 months later both developed the skin tumours with highly malignant histological picture. In 1 patient at the tumour stage the skin tumours were spreading during the PUVA treatment. 3 patients at the first or eczematoid stage cleared completely or partially by PUVA treatment.

Adult↗

[Rare tumor of the cavum (mycosis fungoides)].

A case of mycosis fungoides initiating in the cavum is reported. Results of biopsy of specimens taken from this region were negative, and diagnosis was established only after the appearance of lymph node and cutaneous lesions. The etiology is discussed, particularly in relation to the possible viral nature of the acquired immunodeficiency sundromes and more especially the "gay syndrome".

Adult↗

Electron beam therapy of mycosis fungoides.

Sixteen patients with mycosis fungoides were treated with a 3.3 MeV skin electron beam to a dose of 30 Gy over 40 days. Nine patients achieved a complete remission which was generally short. Only two patients remained free of disease one year following treatment. All patients received palliative benefit from treatment, but no significant increase in survival can be anticipated.

Adult↗

[Clinical characteristics of mycosis fungoides and Sézary syndrome].

Mycosis fungoides and Sézary syndrome are typical representatives of malignant lymphomas of the T-series affecting the skin. Both affections have a varied clinical picture, a different spectrum of histological and laboratory findings and lack a uniform prognosis. In the submitted review the authors discuss in more detail all mentioned aspects of the disease and draw attention to new therapeutic possibilities.

Humans↗

Role of activated T lymphocytes in mycosis fungoides.

A patient with mycosis fungoides was treated effectively with the immunosuppressant cyclosporine A. Excretion of urinary neopterin, a marker of activation of the cellular immune system, fell immediately at the start of therapy. This result argues in support of a central role of activated T lymphocytes in this disease as has already been proposed by other authors.

Adult↗

[Disseminated mycosis fungoides].

A case of mycosis fungoides (MF) that began with cutaneous eritemato-papular lesions leading to death with neurological symptoms is reported. Cutaneous histophatology was typical to MF and the liquoric citology showed Sézary cells. Necropsy evidenced extra-cutaneous dissemination of the tumor involving lymphnodes, heart, digestive system, bladder, liver, bone marrow and leptomeninges. The uncommon clinical manifestations and evolution are discussed.

Adult↗

Follicular mycosis fungoides: successful treatment with oral bexarotene.

Follicular mycosis fungoides, a subtype of cutaneous T-cell lymphoma, is often difficult to treat. We present a case of a female with follicular mycosis fungoides who showed an excellent response to low-dose (150 mg/m2) oral bexarotene (Targretin). To our knowledge, this is the first reported case of follicular mycosis fungoides demonstrating a response to bexarotene.

Administration, Oral↗

Squamous cell carcinoma appearing in X-ray-treated mycosis fungoides.

Two patients with mycosis fungoides developed a squamous cell carcinoma of the skin on the neck. Upon verification by routine biopsy testing of material taken from a suppurating infiltrate of the neck, both patients were treated intermittently with X-rays. Both had developed the squamous cell carcinoma on a sun-exposed area. Patient 1 had been treated with Grenz-ray irradiation totalling 10 kilovolt (kV) 4400 rad. and Dermopan step IV (50 kV) 5200 rad.; while patient 2 recieved altogether: Grenz-rays 4600 rad., Dermopan step IV 400 rad., and soft X-rays (150 kV) 800 r. The latency period was, respectively, 3 and 10 years.

Carcinoma, Squamous Cell↗

Staging lymphography with respect to lymph node histology, treatment, and follow-up in patients with mycosis fungoides.

Extracutaneous involvement in mycosis fungoides (MF) is associated with a poor prognosis. Therefore, a pretreatment staging procedure is used to determine the extent of the disease. As part of the staging procedure lymphography was performed in 36 patients with MF. The radiographic findings and the correlation of these findings with the histopathologic changes in lymph nodes and the clinical course are described. Lymphography in MF shows a variety of abnormalities. However, these abnormalities correlate in only 61% of the patients with the extent and the clinical course of the disease. It is concluded that lymphography is not a useful clinical tool in the staging of MF.

Adult↗

Lymphomatoid papulosis associated with mycosis fungoides: clinicopathological and molecular studies of 12 cases.

The association of mycosis fungoides and a primary cutaneous CD30+ lymphoproliferative disorder has been reported and probably represents different clinical aspects of a unique T-cell monoclonal expansion. In this study, 12 patients (6 men and 6 women) presented with lymphomatoid papulosis and mycosis fungoides. A TCRgamma gene rearrangement study was performed by an automated high-resolution PCR fragment analysis method on skin biopsy specimens taken from the different clinical lesions in each patient. An indolent clinical course was observed in the majority of patients. T-cell clonality was identified in 7 of 12 lymphomatoid papulosis lesions (58%) and in 6 skin biopsies of plaque stage mycosis fungoides (50%). In each individual case, where T-cell clonality was detected, both mycosis fungoides and lymphomatoid papulosis specimens exhibited an identical peak pattern by automated high-resolution PCR fragment analysis, confirming a common clonal origin. Only one case showed a clonal TCRgamma rearrangement from the lymphomatoid papulosis lesion, which could not be demonstrated in the mycosis fungoides specimen. The demonstration of an identical clone seems to confirm that both disorders are different clinical manifestations of a unique T-cell monoclonal proliferation. Our results also seem to confirm that the association of mycosis fungoides with a primary cutaneous CD30+ lymphoproliferative disorder usually carries a favourable prognosis.

Adult↗

Risk of secondary cutaneous malignancies in patients with long-standing mycosis fungoides.

BACKGROUND: Patients with mycosis fungoides (MF) are frequently treated with UV light and psoralen (PUVA), nitrogen mustard, and electron beam irradiation, modalities known to predispose persons to development of cutaneous malignancies. OBJECTIVE: We assessed the relation between these therapeutic modalities and the development of secondary cutaneous malignancies. METHODS: We reviewed the charts of all patients observed during the past year. RESULTS: We found that 7 of 71 patients had cutaneous neoplasms in an average follow-up time of 8.3 years. Orthovoltage radiation was used in five of seven cases and PUVA in four of seven. Five of seven patients had multiple neoplasms. CONCLUSION: The risk of the development of second malignancies from the treatment of MF is relatively small and appears to be related to the type of therapy.

Carcinoma in Situ↗

[Photochemotherapy in mycosis fungoides].

Nineteen patients with mycosis fungoides (m.f.), without involvement of lymph nodes and/or internal organs, were treated with oral photochemotherapy (PUVA). After four to five weeks of PUVA therapy (four irradiations/week) complete remission of erythematous and infiltrative plaques occurred; tumorous m.f. lesions also responded to treatment but required longer treatment times. After complete resolution of m.f. lesions the patients were controlled regularly, the observation periods ranging from 6 to 27 months. When recurrences occurred the initial treatment schedule was resumed. Recurrences, more often seen in the tumorous m.f. stage, responded to PUVA equally well as the initial lesions. PUVA therapy of m.f. is thus more effective than conventional UVB-irradiation and less problematic than treatment with cytotoxic agents or ionizing radiation. Present experience indicates that PUVA represents the treatment of choice in early stage m.f.

Adult↗

Mycosis fungoides: a dermatological masquerader.

Mycosis fungoides (MF), a low-grade lymphoproliferative disorder, is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumours or erythroderma. Diagnosis of MF can be difficult due to highly variable presentations and the sometimes nonspecific nature of histological findings. Molecular biology has improved the diagnostic accuracy. Nevertheless, clinical experience is of substantial importance as MF can resemble a wide variety of skin diseases. We performed a literature review and found that MF can mimic >50 different clinical entities. We present a structured framework of clinical variations of classical, unusual and distinct forms of MF. Distinct subforms such as ichthyotic MF, adnexotropic (including syringotropic and folliculotropic) MF, MF with follicular mucinosis, granulomatous MF with granulomatous slack skin and papuloerythroderma of Ofuji are delineated in more detail.

Diagnosis, Differential↗

Electron-beam therapy for mycosis fungoides.

The lesions of mycosis fungoides are radiosensitive and because extensive areas of the body are usually involved, the treatment of choice is with the electron beam. Since 1971, a fractionation scheme of weekly doses for irradiation of the entire skin surface has been practiced with a 6 MeV linear accelerator that generates 3.5 MeV electrons. One hundred and five patients were treated in this way by delivering 400 rads once weekly for six consecutive weeks. All patitents enjoyed complete disappearance of the skin lesions at once. Of the 62 patients whom we were able to follow-up after electron-beam therapy, for periods ranging from one to nearly seven years (median, two years), the duration of remission was one-half to nearly five and a half years (median, one and one-half years). The treatment was well tolerated and no unusual skin reactions or hematopoietic depression occurred.

Adult↗