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Laryngeal mycosis fungoides.

Mycosis fungoides presented as a tumor of the arytenoid cartilage and epiglottis of the larynx of an 80-year-old female. The tumor was initially interpreted as an undifferentiated small cell malignant neoplasm and treated with radiation. Two years later the patient developed widespread cutaneous involvement with mycosis fungoides, including mycosis fungoides bullosum. The mycosis fungoides progressed to death over the next two years. At autopsy widespread visceral involvement was present. The larynx was extensively infiltrated with mycosis fungoides, and the histology was similar to the original laryngeal biopsy. Although mycosis fungoides is generally thought of as a cutaneous disease, it may rarely present in a squamous epithelium-lined organ other than the skin.

Aged

Topical chemotherapy of mycosis fungoides.

Mycosis fungoides is a T-cell lymphoma which is often localized to the skin in the early stages. Untreated, the process eventually progresses through eczematous, plaque, and tumor stages to systemic involvement. Its course, however, is unpredictable. Topical chemotherapy is effective in early stages of mycosis fungoides. Possibly prognostic benefits can occur from the early use of these agents. Nitrogen mustard and BCNU, both alkylating agents, have been used topically to control the disease. A dermatitis may develop in persons treated with nitrogen mustard but systemic side-effects are rare. However, BCNU may rarely lead to marrow depression when used topically. The use of these agents in mycosis fungoides is discussed herein.

Administration, Topical

Status report of 376 mycosis fungoides patients at 4 years: Mycosis Fungoides Cooperative Group.

The frequency and prognostic importance of various characteristics of patients registered by the Mycosis Fungoides Cooperative Group between November 1974 and December 1977 are reported. Variables which were considered include demographic and historical factors, symptoms, extent of disease, and other physical findings. A staging system which is based on the extent of skin involvement and the number of nodal sites clinically involved is described. Finally, a description of therapeutic results to date for patients randomized into Mycosis Fungoides Cooperative Group protocols is presented.

Adult

Epipodophyllotoxin (VP-16-213) in mycosis fungoides: A report from the Scandinavian mycosis fungoides study group.

Epipodophyllotoxin (VP-16-213) was administered to 9 patients with mycosis fungoides in various stages, most of them in the advanced tumour stage. In 4 of the patients VP-16 was combined with cyclophosphamide. VP-16 alone or in combination with cyclophosphamide was capable of inducing remission initially in all cases, complete in 2, partial in 3 and improvement to a lesser degree in the remaining 5 patients, but it was unable to maintain the remission. The induced remission has to be upheld by other agents, possibly added to VP-16.

Adult

Tumour stage of mycosis fungoides treated with bleomycin and methotrexate: report from the Scandinavian mycosis fungoides study group.

The Scandinavian Mycosis Fungoides Study Group have treated 19 patients with mycosis fungoides in tumour stage by systemic chemotherapy. Nine patients were treated with Bleomycin 15 mg i.m. twice weekly for 7 weeks and 10 patients with the same dose of Bleomycin in combination with Methotrexate 15 mg i.m. per m2 body surface each week for 7 weeks. No maintenance treatment was given. The immediate therapeutic effect of Bleomycin alone was considered good in half of the patients. Bleomycin and Methotrexate together produced a better initial effect. In both treatment series the remission was short-lived in the absence of maintenance therapy. The mortality rate was high, especially in combination treatment. Lethal complications occurred in 6 patients during the treatment, 3 of which were thromboembolic, one pancytopenia, one lung fibrosis with pulmonary insufficiency, and one bronchopneumonia. The conclusion is that these two forms of therapy cannot be recommended.

Adult

Mycosis fungoides plaque stage treated with topical nitrogen mustard with and without attempts at tolerance induction: report from the Scandinavian mycosis fungoides study group.

The Scandinavian Mycosis Fungoides Study Group has treated 21 patients with mycosis fungoides in plaque stages with topical, whole-body application of nitrogen mustard, 20 mg in 40 ml water per square metre. Ten patients were treated after previous attempts at intravenous tolerance induction ad modum van Scott & Kalmanson and eleven without. Complete remission was initially achieved in 10 patients and partial remission in 9 patients. Contact dermatitis to nitrogen mustard developed in 2/10 after tolerance induction and in 1/11 without tolerance induction. It is concluded that topical, whole-body application of nitrogen mustard gives high remission rates. In this series, however, many relapses occurred, due to inadequacy of the maintenance treatment. Tolerance induction has not been found of any value.

Administration, Topical

Chromosomes and B and T cells in mycosis fungoides.

Because mycosis fungoides (MF) and Sézary syndrome (SS) share several features, some investigators have considered them to be different stages of the same disease. Others view them as separate entities. Cytogenetic studies in four typical MF patients showed that the chromosomal abnormalities were different from those reported in SS patients, and the abnormalities were found with almost equal frequency in both B and T cells. In addition, we found the frequencies of the T cells in six of the eight peripheral blood determinations in three patients were within normal range. These observations support the possibility that MF and SS are two separate disease entities.

Aged

[Morphology and monocytopoesis of mycosis fungoides].

Nature and nosology of mycosis fungoides have to be reconsidered because there is now evidence of the t-cell nature of the atypical lymphoid cells within the mycoside infiltrate. Therefore the concept of the reticulum cells in the pathogenesis of mycosis fungoides must be corrected. On the ground of morphological and immunological reasons these cells do not exist in the dermis at all. These new findings are the basis of our morphological study in patients with mycosis fungoides. Concentrating on the polymorphous lymphohistiocytic cells, which cannot be classified further by the method of paraffin-thin sections, light microscopically, the exact differentiation is only possible with semi-thin-sections. With this technique there can be made visible the typical features of nuclei of mycosis fungoides cells. The mycosis fungoides cells are characterized by a large nucleus with hyperconvoluted nuclear membranes, prominent nucleoli, irregular distribution of heterochromatin and sparse cytoplasma. The monocytopoiesis, scheduled by the relative number and the 3H-thymidine labeling indices of promonocytes and the activity of naphthol-AS-D-chloroacetate esterase in blood monocytes was markedly increased. These findings are of special interest, because macrophages are not only able to phagocyte but also play a crucial role in immunology. The elevated monocytopoiesis in all stages of mycosis fungoides points to a stimulation of the immunologic system. This supports the hypothesis of a persistent antigen, which stimulates by means of the functional intact monocyte-macrophage system the lymphocyte system leading to a permanent transformation with augmentation of these immunoblasts (mycosis fungoides cells).

Humans

Lymphography in the assessment of mycosis fungoides.

Extracutaneous manifestations of mycosis fungoides imply a bad prognosis and are a major cause of death. Benign dermatopathic lymphadenopathy is associated with mycosis fungoides and often precedes lymphomatous infiltration. In this study, 10 patients in the early stages of mycosis fungoides underwent clinical and lymphographical examinations. In one the lymphoma was already present in lymph nodes. Six had signs of dermatopathic adenopathy which was verified by lymph node biopsy in 5. In one of these the disease later progressed to a malignant lymphoma. The frequent occurrence of lymph node involvement justifies the use of lymphography collaterally with staging laparotomy to determine the presence of pathologic retroperitoneal lymph nodes.

Adult

Bleomycin therapy in mycosis fungoides.

Nine patients with mycosis fungoides in different stages were treated with Bleomycin. Much better results were obtained with this new drug in the six patients with the infiltrative or beginning tumour stage than in the patients in the advanced tumour stage. Complete remission was not seen. In one case the results were objectivized by DNA cytophotometry. The role of Bleomycin in the treatment of mycosis fungoides is discussed. It is concluded that Bleomycin is not the medicament of choice for the treatment of mycosis fungoides.

Aged

Meningeal mycosis fungoides: cytologic and ultrastructural aspects.

Mycosis cells were identified in the pre-morbid cerebrospinal fluid of a patient with neurological symptoms and mycosis fungoides (MF). Light and electron microscopic examination at autopsy confirmed leptomeningeal involvement by mycosis fungoides. The cellular morphology of the non-cutaneous infiltrates supports the concept that mycosis fungoides retains a unique histopathology in its dissemination to the viscera. The importance of cerebrospinal fluid cytology in patients with mycosis fungoides is emphasized.

Brain Neoplasms

Cerebriform (Sézary like) mononuclear cells in healthy individuals: a morphologically distinct population of T cells. Relationship with mycosis fungoides and Sézary's syndrome.

The ultrastructural and surface marker characteristics of lymphocytes in human cord blood and peripheral blood of healthy donors were studied with respect to the presence of cerebriform mononuclear cells similar to those occurring in the dermal infiltrate of patients with mycosis fungoides (mycosis cells), and the skin infiltrate and peripheral blood of patients with Sézary's syndrome (Sézary cells). Cerebriform monuclear (Sézary-like) cells are characterized by a high nucleus-cytoplasm ratio, deep and narrow nuclear identations, condensed chromatin at the nuclear membrane and cytoplasm poor in organelles. Of the lymphoid cells in human cord blood and peripheral blood of healthy donors 6.7 and 8.7% respectively proved to be cerebriform mononuclear cells. Since these cells invariably form E-rosettes they are part of the T-cell population in healthy individuals. The finding of similar cells in the skin infiltrate of patch test areas of patients allergic to rubber, formalin and peruvian balsam--an expression of cellular immunity mediated by T-cells--suggests that these cells are reactive T cells. Not all (up to 85%) of the cerebriform mononuclear cells in patients with mycosis fungoides and Sézary's syndrome have T-cell membrane characteristics as shown by E-rosette formation. This suggests the presence of two populations of cerebriform mononuclear cells in mycosis fungoides and Sézary's syndrome. The relationship of cerebriform T cells as seen in healthy individuals with cerebriform or atypical mononuclear cells occurring in the Sézary syndrome and mycosis fungoides is discussed.

Adolescent

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

Adriamycin therapy in advanced mycosis fungoides.

Thirteen patients with advanced mycosis fungoides received induction therapy with Adriamycin, 60/m2 I.V. repeated at 21-day intervals. Ten patients had extensive skin tumors; all patients had lymph node enlargement with mycosis fungoides involvement in eight; four patients had biopsy-proven visceral involvement. Only two patients had received no prior therapy. The overall response rate with Adriamycin therapy was 85% with three patients (23%) achieving a biopsy-proven complete remission and five patients (39%) partial remissions. The median number of courses to maximum response was two (range two to four). The principle toxicity was myelosuppression, but this was not severe and the entire group received more than 90% of the intended doses of Adriamycin. One patient developed probable Adriamycin cariotoxicity. Maintenance therapy for patients achieving a remission was methotrexate 15 mg/m2 I.M. twice weekly and cyclophosphamide 750 mg/m2 I.V. every 21 days. The median duration of complete remission was 32+ weeks (range 16+-40+ weeks) while the median duration of partial remission was 18 weeks (range 8-111+ weeks). Adriamycin has proven to be an effective induction agent in the treatment of advanced mycosis fungoides and its incorporation into combination chemotherapy regimens is warranted.

Bone Marrow

Subcutaneous mycosis fungoides.

In five cases of mycosis fungoides, previously treated with electron-beam therapy, subcutaneous nodules developed. Clinically, these lesions were thought to be epidermoid cysts or lipomas, but on biopsy were discovered to be subcutaneous infiltrates, three of which were diagnosed as mycosis fungoides. The other two specimens showed only a nonspecific subcutaneous infiltrate. There is no ready explanation for the appearance of these lesions, but it is speculated that they may be the result of inadequate penetration of the electron beam to the depth at which some atypical cells may originally have been located. Patients with mycosis fungoides who develop unusual subcutaneous nodules should be fully investigated so that appropriate and adequate therapy may be initiated.

Adult

Gingival involvement in mycosis fungoides: report of case.

A case of mycosis fungoides of the gingiva is reported. Mycosis fungoides is a lymphoproliferative disease that primarily affects the skin. Systemic dissemination is a distinct aspect of the natural course of mycosis fungoides and usually involves the lymph nodes, spleen, and liver. The diagnosis of mycosis fungoides depends on the presence of a variety of atypical cells, including small and large variants of the mycosis cell with an infiltrate of polymorphous inflammatory cells in both the dermis and epidermis. Various modes of treatment have been used. In the current case, remission was achieved with radiation therapy.

Aged

Leptomeningeal mycosis fungoides.

Central nervous system involvement with mycosis fungoides complicated the clinical course of a patient at a time when his skin was clinically free of disease following systemic chemotherapy. A leptomeningeal syndrome of blurred vision and papilledema, and confusion progressing to coma, was associated with elevated spinal fluid pressure and abnormal spinal fluid cells morphologically similar to those seen in the Sezary syndrome. The symptoms were dramatically reversed by intrathecal methotrexate, brain irradiation, and steroids. Mycosis fungoides recurred in the skin, in the spinal fluid, and in both eyes. Despite continued systemic and intrathecal chemotherapy, the patient died from mycosis fungoides. This is the second patient reported with meningeal mycosis fungoides.

Antineoplastic Agents