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[Mycosis fungoides. A review of the clinical picture, treatment and course in 107 patients].

During the period 1972 till the end of 1987, 107 patients with the diagnosis of mycosis fungoides or cutaneous T-cell lymphoma were examined and treated in the Department of Dermatology, Marselisborg Hospital. This disease belongs to the group of non-Hodgkin T-cell lymphomata. The diagnosis is based on the occurrence of red, scaly plaques in the skin associated with itching or tumours in the skin and, simultaneously, of a pleomorphic infiltrate consisting of CD4-positive T-lymphocytes which show characteristically exocytoses in the epidermis with subsequent formation of Pautrier's microabscesses. The disease may progress with spread to the regional lymph nodes where lymphomata develop. Treatment is initially local with employment of chlormethin ("nitrogen mustard gas") and this treatment can maintain the patients in remission for prolonged periods. In cases with spread to lymph glands or in particularly aggressive forms with tumour formation in the skin, combined chemotherapy is administered (prednisone, cyclophosphamide, etretinate and bleomycin). Thirty-eight of the patients were in stage I in which a clinical suspicion of mycosis fungoides was present but where the histological changes were insufficient to confirm the diagnosis. IVa and ten in stage IVb. The age at the onset of the symptoms was from 59 to 64 years (median values) for the various stages. Stages I and II had approximately 80% five-year survival, while the stages with more extensive spread had approximately 50% survival. The etiology of the disease is unknown but, during recent years, certain evidence has been found suggesting that activation of a retrovirus in the epidermis may be a contributory factor.

Antineoplastic Combined Chemotherapy Protocols↗

[Comparison of the results obtained with ultraviolet radiation and photochemotherapy in the early stages of mycosis fungoides].

The Authors have performed a retrospective study, comparing the results obtained in two groups of patients affected by Mycosis Fungoides at early stages. Thirty-four patients were treated with ultraviolet rays, 32 with photochemotherapy. The patients, in the first stages of the disease, classified on the basis of TNM system, were treated with UVR or PUVA according to standard schedules. The results obtained in the two groups were the following: 1. UVR treated group: complete remission 32%, partial remission 65%, no results 3%; 2. PUVA treated group: complete remission 84%, partial remission 16%. These data show the evidence that photochemotherapy is undoubtedly more advantageous than simple phototherapy in inducing complete remission of early Mycosis Fungoides. UVR therapy still presents, however, as a worthwhile non-aggressive therapeutic choice when the disease has not a progressive character or when photochemotherapy has serious contraindications.

Follow-Up Studies↗

[Disordered keratinocyte differentiation in epidermal tumors and mycosis fungoides].

The immunocytochemical method with monoclonal antibodies to human and murine epidermis basal-cell antigen (BCAg) has been used in studies on the disorders in keratinocyte differentiation in epidermal tumors and mycosis fungoides. The fluorescence intensity augmented and BCAg distribution in the cytoplasm grew more diffuse as the degree of the tumor cell differentiation lowered. In mycosis fungoides the BCAg reaction has been positive first in the suprabasal layers; as the disease progressed, the fluorescence has involved the entire epidermis. The BCAg test is a sensitive tool for estimating the epidermal cell maturity in processes associated with impaired differentiation of keratinocytes.

Antibodies, Monoclonal↗

Topical nitrogen mustard in early mycosis fungoides. A 12-year experience.

A 12-year experience in thirty-three patients suffering from early mycosis fungoides in plaque stage confirms the effectiveness of topical nitrogen mustard therapy. Fourteen patients were in complete remission at the latest time of observation and 7 in partial remission. The probability of freedom from relapse was approximately 50% after 6 and 12 years. Three deaths attributable to mycosis fungoides were recorded. Three patients had to discontinue treatment due to contact dermatitis to nitrogen mustard. Specific precautions were undertaken in order to protect personnel handling the drug. No damaging or toxic effects were observed among staff personnel and no hematological side-effects were observed among the patients. The treatment as a whole was well tolerated.

Administration, Topical↗

Reactivity of T cells in mycosis fungoides exhibiting marked epidermotropism with the monoclonal antibody HML-1 that defines a membrane molecule on human mucosal lymphocytes.

Twenty-eight cases of cutaneous T cell lymphomas of mycosis fungoides type, 8 of which showed a marked epidermotropism, were investigated for their reactivity with the monoclonal antibody HML-1. This reagent selectively recognizes human benign and malignant intestinal T lymphocytes, but not benign or malignant lymph node T cells. In all cases, the majority of nonepithelium-associated T cells present in the corium were HML-1 negative. In the eight cases with marked epidermotropism, the intraepidermal (IED) T cells were HML-1 positive in three cases, and HML-1 negative in the remaining five cases. The HML-1 positive IED T cells tended to be localized preferentially in the basal layers, whereas the HML-1 negative IED T cells were more diffusely distributed throughout all epidermal cell layers or formed intra-epidermal aggregates. These findings provide further evidence that mycosis fungoides is not a single entity, but represents a group of neoplasms originating from different T cell subsets that have in common their tropism to epidermis and/or dermis, and thus have a similar clinical presentation.

Antibodies, Monoclonal↗

Evidence for HTLV-I associated with mycosis fungoides and B-cell chronic lymphocytic leukemia.

Human T-cell lymphotropic virus type I (HTLV-I) is a human retrovirus that can transform T-helper lymphocytes and is etiologically associated with adult T-cell lymphoma/leukemia. Mycosis fungoides represents a primary cutaneous lymphoma of helper T-cell origin, while chronic lymphocytic leukemia is generally considered to be a neoplastic B-lymphocyte disorder. Our patient had HTLV-I with coexistent mycosis fungoides and B-cell chronic lymphocytic leukemia. The concurrent lymphoid proliferations may represent HTLV-I-associated abnormalities of immunoregulation.

Aged↗

Isotretinoin and cutaneous helper T-cell lymphoma (mycosis fungoides).

Retinoids, including isotretinoin, have demonstrated antiproliferative and antineoplastic activity in laboratory and clinical trials. In a phase II trial, 25 patients with extensive mycosis fungoides were evaluated for response to isotretinoin. There was a 44% (11 patients) objective clinical response rate with three clinical complete responses without concomitant evidence of pathologic clearing of the disease. An additional 24% (six patients) showed a minor degree of clinical improvement. The median time to response was two months (range, 0.5 to eight months) and the median response duration was eight months or longer (range, one to 25 months). Chronic toxic reactions consisted primarily of drying of the skin and mucous membranes and resulted in dose reduction in the majority of patients. It is concluded that isotretinoin produces significant clinical benefit to some patients with mycosis fungoides.

Adult↗

Treatment of mycosis fungoides lymphoma: effectiveness of infusions of methotrexate followed by oral citrovorum factor.

Eleven patients with stage II or III mycosis fungoides lymphoma were treated with a regimen consisting of iv infusions of methotrexate (MTX) (60-240 mg/m2) administered sequentially and oral citrovorum factor. All 11 patients experienced a good to excellent response. Complete remissions were induced in seven of the 11 patients. In most cases remissions were sustained with weekly administration of low-dose (25--50 mg) MTX. Adverse drug reactions, such as leukopenia, anemia, and mucositis, were minimal in degree. Some unique adverse reactions included skin ulcerations (three patients), a leukocytoclastic angiitis (one patient), and painful hands and feet during MTX infusions (two patients). Our experience with this regimen has led us to conclude that it is safe and very effective in the treatment of stages II and III of mycosis fungoides.

Administration, Oral↗

Coexisting Hodgkin's disease and mycosis fungoides. Immunohistochemical proof of its existence.

Hodgkin's disease and mycosis fungoides have been rarely reported in the same patient. This coexistence has been debated in the medical literature. We studied such a patient and report, to our knowledge, the first immunophenotypic evidence for such a coexistence. Reed-Sternberg cells and their variants stained with anti-Leu-M1, Hefi-1, anti-Tac, anti-HLA-DR, and OKT9, but were negative for T cell markers 3A1, Leu-1, Leu-2a, and Leu-3a, a phenotype typical of Hodgkin's disease; infiltrating small lymphocytes were predominantly T cells and were phenotypically normal. In the skin lesions, cells with the phenotype of Hodgkin's disease were not present; the infiltrate was composed of helper T lymphocytes that were 3A1-negative, a phenotype characteristic of the malignant cells of mycosis fungoides. Unexpectedly, a dermatopathic lymph node from the same patient showed the presence of the Leu-M1 antigen on the majority of normal-appearing interdigitating reticulum cells; this was not the case with control dermatopathic lymph nodes from patients without a malignancy. The significance, implications, and possible interrelationships of the findings are discussed.

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↗

[A combination of mycosis fungoides and chronic myeloid leukemia. Apropos of a case].

The coexistence of a T-cell lymphoma with a myelodysplatic syndrome seems to be exceptional. In the case reported here the diagnostic problems raised by the appearance of cutaneous nodules in a patient with chronic myeloid leukaemia (CML) were solved by histo-immunological examinations. A 70-year old male patient had been presenting since 1976 with a psoriasis-like skin disease. He was first seen at the Argenteuil hospital in 1984. Physical examination showed psoriasiform finger-like erythemato-squamous lesions, infiltrated plaques and an ulcerated tumoral swelling of the right elbow. A diagnosis of mycosis fungoides was made on histological and immunological examination results. At histology, this epidermotropic lymphoma was peculiar in that the atypical infiltrate was clearly centred on vessels. Electron microscopy confirmed that the vascular walls were invaded by the mycosis cells. Additional examinations showed hyperleucocytosis and myelaemia which were rapidly attributed to a chronic myelocytic leukaemia since the Philadelphia chromosome was present and the leucocytes had a low alkaline phosphatase score. Bone marrow biopsy disclosed a myeloproliferative syndrome of the CML type. Biopsy of a right axillary lymph node showed myelocytic infiltration associated with dermopathic lymphadenitis. There were no circulating Sezary cells, and a search for extension proved negative. From May, 1984 to June, 1985 the patient's CML was treated with busulfan which produced blood and bone marrow remission. The skin lesions were treated first with mechlorethamine, then with topical corticosteroids. Superficial electron therapy was applied to the tumoral lesions.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Fine-needle aspiration biopsy of mycosis fungoides presenting as an ulcerating breast mass.

A patient with previously diagnosed Hodgkin's disease presented with ulcerating lesions on her scalp and breast. Fine-needle aspiration of the breast lesion revealed convoluted cells consistent with mycosis fungoides. Review of the patient's previously obtained lymph node biopsy specimens revealed a diagnosis of mycosis fungoides rather than Hodgkin's disease. Better communication between hospitals, pathologists, and clinicians may have prevented the erroneous original diagnosis of Hodgkin's disease in this patient.

Adult↗

Recombinant leukocyte interferon alfa-2a in the treatment of mycosis fungoides.

This study was designed to evaluate the efficacy and tolerability of recombinant leukocyte interferon alfa-2a (Hoffmann-LaRoche) as single agent in patients with histologically confirmed Mycosis Fungoides. The protocol consisted of a 12 week induction with subcutaneous interferon, escalating from 3 up to 18 million units daily, and a 6 or 9 month maintenance phase for complete or partial responses, respectively, with 18 million units 3 times weekly. 12 patients are evaluable: 5 are in complete remissions, 6 are partial remissions, and one had disease progression. Alfa-2a interferon was well tolerated: only 3 patients had WHO grade IV organ toxicity. Our study documents that recombinant leukocyte alfa 2a is a highly active agent in untreated patients with Mycosis Fungoides. Finally, the dose schedule chosen in this study allows alfa-2a interferon administration on an outpatient basis.

Adult↗

[Mycosis fungoides and Sézary syndrome. A retrospective study of 60 patients with correlation between histologic classification and survival time].

47 patients suffering from mycosis fungoides and 13 with Sézary's syndrome were retrospectively studied with regard to prognosis in relation to the histologic substrate of cutaneous lesions. On account of the wide spectrum of T-cell cytomorphology as well as the pattern and composition of the infiltrate, we distinguished 4 histopathological types: small cell lymphoid type (45%), mixed cell polymorphic type (40%), large cell type (immunoblastic and pleomorphic, 10%), and medium-sized cell type (5%). Statistical analysis revealed survival rates of 10 years in 68.3% for the small cell lymphoid type and in 30.3% for the mixed cell polymorphic type. All patients showing the large cell type died within 6 years after diagnosis; those bearing the medium-sized cell type died within 2 years after diagnosis. The forms of mycosis fungoides showing the last two types of infiltrate may be classified as lymphomas of extreme malignancy. The different histological features are not only significant with regard to the diagnosis but also permit prognostic estimation before systemic signs have become evident.

Adult↗

Hypopigmented mycosis fungoides. Speculations about the mechanism of hypopigmentation.

Hypopigmented, nonatrophic macules are an unusual manifestation of lesions of early mycosis fungoides. Presented herein is a patient with such lesions and an unusual mask-like hypopigmentation of the face. We review both the clinical and microscopic findings in this patient and in previously reported patients with hypopigmented lesions of mycosis fungoides. Finally, speculation is made as to the mechanisms of the hypopigmentation.

Adult↗

A case-control study of possible causative factors in mycosis fungoides.

A detailed case control study was carried out on 53 patients (33 males and 20 females) with histologically proven mycosis fungoides and on an age- and sex-matched control population. Possible causative factors investigated included occupation, recreation, and exposure to petrochemicals, pesticides, insecticides, and potential carcinogens. Exposure to plants of the Compositae family, tanning history, and chronic sun exposure were also investigated, as were smoking history, drug ingestion history, and other skin disease. Personal and family histories of other malignancies were also investigated. The only statistically significant difference to emerge was that the patients with mycosis fungoides had significantly more family history of atopic dermatitis. In view of the absence of any significant difference between patients and controls with regard to personal history of atopic dermatitis, this difference may be the result of multiple statistical testing rather than a phenomenon of true biological significance.

Adolescent↗

[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↗