[Lymphoma fungoides (mycosis fungoides); nitrogen mustard therapy].
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Humans↗
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Results for “MYCOSIS FUNGOIDES”
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Pagetoid reticulosis, epidermotropic mycosis fungoides and mycosis fungoides: a disease spectrum.
Using a standard technique involving monoclonal antibodies against T-cell subsets, we have shown that almost all the infiltrating T-cells in the epidermis of a patient with Pagetoid reticulosis (PR), one with epidermotropic mycosis fungoides (EMF) and one with poikiloderma atrophicans vasculare (PAV), were OKT8 positive (presumed cytotoxic/suppressor) T-cells. The infiltrating T-cells in the epidermis of a patient with limited plaque stage mycosis fungoides (MF), however, were almost exclusively Leu 3a-positive (presumed helper/inducer) T-cells as is usually found in this condition. The keratinocytes in the patients with PR, EMF and PAV were HLA-DR-positive whilst those in the patient with MF were HLA-DR-negative. We consider these four diseases to be part of the spectrum of mycosis fungoides, the first three conditions representing the early or benign end of the spectrum.
Adult↗
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↗
Milia during treatment of mycosis fungoides: follicular mycosis fungoides?
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Acitretin↗
Chronic cutaneous lupus erythematosus mimicking mycosis fungoides.
Mycosis fungoides, which is characterized by a malignant infiltrate of T lymphocytes involving the epidermis, can be confused with other inflammatory skin diseases. We report the case of a patient with skin lesions containing an infiltrate of atypical lymphocytes with epidermotropism. This patient's condition was initially diagnosed as mycosis fungoides. Repeated biopsy samples had the histologic features of chronic cutaneous lupus erythematosus. The patient had a strongly positive antinuclear antibody response and the clinical lesions responded to hydroxychloroquine, however, and these findings led to an altered diagnosis. Other disorders that either clinically or histologically mimic mycosis fungoides are reviewed, and the diagnostic evaluation of patients in whom mycosis fungoides is suspected is summarized. Chronic cutaneous lupus erythematosus should be added to the list of diseases that can mimic mycosis fungoides.
Antibodies, Antinuclear↗
p16(INK4a) gene alterations are frequent in lesions of mycosis fungoides.
Mycosis fungoides is usually an indolent disease that, after a variable period of time in a stable phase, evolves into a tumoral form with aggressive behavior. The molecular events that mark this progression remain essentially unknown to date, and this prompted us to investigate the hypothetical role of p16(INK4a) silencing in mycosis fungoides progression. We analyzed the three most frequent mechanisms of inactivation of the p16(INK4a) gene (deletion, promoter hypermethylation, and mutation) in nine cases with patch/plaque and tumoral lesions of mycosis fungoides. The existence of alterations was investigated by microsatellite analysis, methylation-specific polymerase chain reaction, and direct sequencing. Alterations of the p16(INK4a) gene were found in four of nine of the plaque lesions (hypermethylation in three samples and allelic loss in one sample) and seven of nine in the tumor lesions (hypermethylation in five samples and allelic loss in three samples). No case presented point mutations. Although a higher incidence of p16(INK4a) hypermethylation was observed in the cases that failed to achieve a complete remission, the limited size of our series prompted us to evaluate this finding cautiously. The results of this study therefore showed a common genetic alteration that is found more frequently in tumoral lesions than it is in plaque lesions of mycosis fungoides. It also offers data that could suggest a relationship between p16(INK4a) hypermethylation and unfavorable clinical outcome. Broader studies are needed to confirm both relationships.
Adult↗
Staging procedures in mycosis fungoides.
Mycosis fungoides (MF) in deep-seated lymph nodes, spleen or liver appears to be associated with a lack of response of the disease to topical external therapy. Fourteen patients with mycosis fungoides were clinically staged and had a staging laparotomy. Mycosis fungoides was found in the lymph nodes of 3 of these patients, in the spleen in 4 and in the liver in 2 cases. Patients with adenopathy, or with splenomegaly or abnormal spleen scans, should be considered for surgical staging to determine the extent of extracutaneous disease. The documentation of extracutaneous MF becomes necessary in the development of new therapeutic approaches.
Adenoids↗
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↗
Recalcitrant tinea corporis as the presenting manifestation of patch-stage mycosis fungoides.
Mycosis fungoides is a cutaneous T-cell lymphoma. Its presence, which denotes an altered immune system, may make treatment of otherwise simple cutaneous infections difficult. In the case presented here, a patient with widespread tinea corporis poorly responsive to several oral antifungals was noted as having a background poikilodermatous slightly scaly eruption. Results of a skin biopsy during therapy with oral antifungal medications showed evidence of tinea corporis; atrophy of the epidermis; a superficial, perivascular, and interstitial lymphocytic infiltrate with numerous atypical lymphocytes; and exocytosis of atypical lymphocytes into the epidermis with formation of microabscesses-findings consistent with the diagnosis of mycosis fungoides. Treatment with PUVA (oral psoralen and UVA light) and oral itraconazole led to long-term remission of the mycosis fungoides and the associated tinea corporis. Immune suppression may have contributed to the recalcitrant nature of our patient's dermatophyte infection. Underlying cutaneous, systemic, or iatrogenic disorders associated with immune dysfunction should be considered in patients with recalcitrant dermatophyte infections.
Female↗
A randomized trial comparing combination electron-beam radiation and chemotherapy with topical therapy in the initial treatment of mycosis fungoides.
Mycosis fungoides is a T-cell lymphoma that arises in the skin and progresses at highly variable rates. Nonradomized studies have suggested that early aggressive therapy may improve the prognosis in this usually fatal disease. We studied 103 patients with mycosis fungoides, who, after complete staging, were randomly assigned to receive either combination therapy, consisting of 3000 cGy of electron-beam radiation to the skin combined with parenteral chemotherapy with cyclophosphamide, doxorubicin, etoposide, and vincristine (n = 52) or sequential topical treatment (n = 51). The prognostic factors were well balanced in the two groups. Combined therapy produced considerable toxicity: 12 patients required hospitalization for fever and transient neutropenia, 5 had congestive heart failure, and 2 were later found to have acute nonlymphocytic leukemia. Patients receiving combined therapy had a significantly higher rate of complete response, documented by biopsy, than patients receiving conservative therapy (38 percent vs. 18 percent; P = 0.032). After a median follow-up of 75 months, however, there was no significant difference between the treatment groups in disease-free or overall survival. We conclude that early aggressive therapy with radiation and chemotherapy does not improve the prognosis for patients with mycosis fungoides as compared with conservative treatment beginning with sequential topical therapies.
Antineoplastic Combined Chemotherapy Protocols↗
Deleted HTLV-I provirus in blood and cutaneous lesions of patients with mycosis fungoides.
Mycosis fungoides, a rare form of cutaneous T cell leukemia/lymphoma, is suspected of having a viral etiology on the basis of certain similarities to adult T cell leukemia, which is associated with human T cell leukemia/lymphoma virus type I (HTLV-I) infection. Cell lines were established from peripheral blood mononuclear cells (PBMC) of an HTLV-I-seronegative patient with mycosis fungoides. DNA hybridization analysis revealed the presence of HTLV-I-related sequences with unusual restriction endonuclease sites. Sequence analysis of subcloned fragments demonstrated the presence of a monoclonally integrated provirus with a 5.5-kilobase deletion involving large regions of gag and env and all of pol. Additional evidence for the presence of deleted proviruses was found by polymerase chain reaction (PCR) amplification of DNA from cutaneous lesions of five other HTLV-I-seronegative patients. The findings suggest that HTLV-I infection may be involved in the etiology of at least certain cases of mycosis fungoides.
Base Sequence↗
Necrotizing vasculitis within cutaneous lesions of mycosis fungoides.
Mycosis fungoides is a T cell lymphoma with a predilection for cutaneous involvement. This paper describes the clinical manifestations and histopathologic features of a case of mycosis fungoides with necrotizing vasculitis localized to the lesions of cutaneous lymphoma. Elevated levels of circulating immune complexes were found in this patient. The large numbers of perivascular malignant helper T lymphocytes may have induced immunoglobulin synthesis, resulting in the formation of these complexes followed by deposition in vessel walls and subsequent necrotizing vasculitis. Possible alternative mechanisms include the presence of anti-T cell antibodies, or cytotoxic effector cells.
Adult↗
Coexistence of pemphigus foliaceus and mycosis fungoides.
Mycosis fungoides was documented in a patient two years after pemphigus foliaceus had been diagnosed and treated with corticosteroids. Eight years later, the patient was found to have stage IV lymphomatous disease with generalized erythroderma and palpable, histologically positive lymph nodes. Hematoxylin-eosin staining of a specimen of erythroderma revealed mycosis fungoides, while direct immunofluorescence of this tissue revealed intercellular IgG deposits diagnostic of pemphigus foliaceus.
Aged↗
Dynamic contrast enhanced perfusion MRI in mycosis fungoides.
Mycosis fungoides is a malignant, cutaneous lymphoma of T-helper (TH or CD4+) cells. At presentation, the disease is usually limited to the skin, with lesions that resemble eczema or psoriasis. Neurologic involvement is uncommon. This case demonstrates the conventional MRI and dynamic contrast enhanced perfusion MRI findings in intracerebral mycosis fungoides. T1-weighted spin echo imaging demonstrated a lesion with slightly decreased signal within the body of the corpus callosum. The lesion was isointense with grey matter on axial T2-weighted imaging. Following administration of contrast, there was patchy heterogeneous enhancement. Multiple relative cerebral blood volume (rCBV) measurements were made and the minimum rCBV was 0.30 with the maximum rCBV being 1.61. The mean rCBV was 0.81 +/- 0.49 (average of 10 measurements and standard deviation).
Aged↗
The head and neck manifestations of mycosis fungoides.
Mycosis fungoides (MF) is a cutaneous T-cell lymphoma that may progress to involve lymph nodes, the spleen, and other visceral organs. We have retrospectively reviewed our 10-year experience with MF at the Johns Hopkins Hospital. Forty-five patients received treatment for MF between 1983 and 1992. Approximately 70% of the patients with MF presented with cutaneous or extracutaneous lesions of the head and neck. The head and neck manifestations, clinical assessment, and treatment of our patient population are presented. Mycosis fungoides is a potentially lethal disease that frequently involves the head and neck region and should be included in the differential diagnosis of cutaneous head and neck lesions.
Antineoplastic Agents↗
The natural history of mycosis fungoides.
Mycosis fungoides is a progressive, fatal disease. Its relationship to lymphoblastomas is definite. No specific treatment is available. Palliation may be brought about by many unrelated modalities.
Humans↗
Solitary skin lesions with histopathologic features of early mycosis fungoides.
Mycosis fungoides (MF) is a cutaneous T-cell lymphoma that usually begins with cutaneous patches that evolve into plaques and tumors. A few recent reports describe a solitary variant of MF distinct from localized pagetoid reticulosis, a disease in which solitary verrucous lesions occur on acral skin. Solitary skin lesions with some of the histopathologic features of MF rarely occur during treatment with several drugs, especially antidepressants or antihistamines. We analyzed the clinicopathologic features of 20 patients with solitary skin lesions showing histopathologic features of patch- or early plaque-stage MF. Eight men and 12 women (mean age 50.6, range 23-82, median 49) had solitary, small erythematous patches or plaques located on the trunk (16 cases, 6 of them on the breast), upper extremities (3 cases), and inguinal region (1 case). Ten patients were treated with one or more drugs; only two of them received antidepressants or antihistamines. Histopathologic examination revealed in all cases a band-like infiltrate in the upper dermis, frequently with epidermotropism of solitary lymphocytes. Atypical lymphocytes were present in a minority of cases. Immunohistology showed a predominance of CD3+ T lymphocytes, in most cases admixed with clusters of CD20+ B-cells. Only a small proportion of the infiltrate was CD8+. Molecular analysis of the rearrangement of the T-cell receptor genes was performed in 16 cases using the polymerase chain reaction (PCR) technique and revealed a monoclonal band in 8 of them. After surgical excision, 2/14 patients had a recurrence near the surgical scar. In 18 patients with complete follow-up data, no evidence of "classic" MF could be observed after a mean follow-up of 31.9 months. Solitary skin lesions with the histopathologic features of MF can be considered as a distinct clinicopathologic entity, probably representing a solitary variant of mycosis fungoides.
Adult↗
[Modern therapy of mycosis fungoides].
Mycosis fungoides (MF) treatment must be chosen only after staging extent of lymphoma. Stage I and II diseases respond best to either topical nitrogen mustard, photochemotherapy (PUVA), or electron beam. Chemotherapy of stage III and IV MF has only limited remission rates. Combinations of therapies and experimental approaches, such as monoclonal antibodies, may give longer survivals.
Antineoplastic Agents↗