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Depressed lymphokine activated killer cell activity in mycosis fungoides. A possible marker for aggressive disease.

Peripheral blood mononuclear cells from 24 patients with mycosis fungoides were used to generate lymphokine activated killer (LAK) cells in vitro by culturing with recombinant interleukin 2. Patients with stage la mycosis fungoides were capable of generating normal levels of LAK cell activity, while patients with more active disease (stages IB to IV) had depressed LAK activity. The ability of these patients' cells to respond in a proliferation assay to various mitogens was similar to that of controls, with the exception of patients in the terminal phase of their illness. Patients with active disease who were unable to generate LAK activity were capable of responding in a proliferation assay to interleukin 2. The results of this study suggest that depressed LAK cell activity in patients with mycosis fungoides may serve as an indicator of a more aggressive disease state.

Adult↗

Mycosis fungoides evolving to myelomonocytic leukemia.

Acute myelomonocytic leukemia, a type of leukemic conversion that may be a manifestation of mycosis fungoides, developed in two patients with fairly typical courses of mycosis fungoides. The cases of five other patients with myelogenous leukemia as the terminal event of mycosis fungoides have been reported in the literature. A relationship to therapy could not be established, although all patients had had irradiation or chemotherapy or both.

Female↗

Granulomatous slack skin: a distinct disorder or a variant of mycosis fungoides?

About 75% of cutaneous lymphomas belong to the group of T-cell lymphomas. Mycosis fungoides is the most common entity in this group. Granulomatous slack skin is a rare form of cutaneous T-cell lymphoma closely related to mycosis fungoides. We present here a patient with areas of lax skin for several years who developed a generalized erythroderma with associated immunoactivation and a deterioration in his general condition. This report discusses clinically and histologically the differential diagnoses, namely granulomatous slack skin and granulomatous mycosis fungoides, and suggests that these 2 disorders are only variants in the broad spectrum of a single disease.

Aged↗

Indications of a considerable decrease in the death rate in mycosis fungoides by PUVA treatment.

PUVA therapy has its roots in ancient India and Egypt and began to come into general use in the highly developed countries in the middle of the 1970's (1). The first reports of PUVA treatment of mycosis fungoides were published in 1976 (2); these were followed by several other studies in the two following years (3-7). Some of the early work on PUVA therapy was carried out in Sweden (8,9), and the modality was in general use in most major clinics by 1977. The dramatic effect on mycosis fungoides of PUVA therapy is well known, but whether the death rate is influenced is not known. For ethical reasons no controlled clinical studies have been performed. Sweden is a highly organized country with reliable death statistics at least for diseases as conspicuous as mycosis fungoides. The purpose of the present study was to provide data on the death rate in mycosis fungoides in Sweden from 1961 to 1990, which we think is relevant to the question whether PUVA treatment decreases the death rate in mycosis fungoides.

Humans↗

Mycosis fungoides with follicular mucinosis displaying aggressive tumor-stage transformation : successful treatment using radiation therapy plus oral bexarotene combination therapy.

Follicular mucinosis is a tissue reaction pattern characterized by mucin deposition with follicular sebaceous units and is found as an idiopathic, primary, benign process (alopecia mucinosa), or as a secondary process due to inflammatory and neoplastic disorders (mycosis fungoides). When associated with follicular mucinosis, mycosis fungoides commonly pursues an aggressive course, often undergoing large-cell transformation, which is associated with resistance to therapy and poor prognosis. We present a case of mycosis fungoides with follicular mucinosis that was treated with incomplete courses of interferon, isotretinoin, and polychemotherapy with subsequent rapid progression to tumor-stage mycosis fungoides with large cell transformation and nodal and bone marrow involvement. In this setting, the patient was treated with local radiation therapy, total-skin electron beam therapy, and therapy and maintenance with the oral retinoid-X-receptor retinoid bexarotene, and achieved a durable complete remission.

Administration, Oral↗

Mycosis fungoides: a retrospective study.

The clinical course of ninety patients with the histopathologic diagnosis of mycosis fungoides is reviewed. An attempt is made to correlate sex, age, of onset, lesion type, and form of therapy with outcome of the disease. The data indicate that mycosis fungoides affects predominantly middle-aged males. Patients developing tumors or erythroderma in middle age or later tend to have a shorter survival. More aggressive therapy is associated with shortened survival. Most deaths were due to unknown or unrelated processes and therapeutic complications. The data support the theory that mycosis fungoides can be a relatively nonaggressive cutaneous lymphoma. In an attempt to treat aggressively, we may be exposing patients to increased mortality.

Adolescent↗

Expression of Leu-8 antigen, a majority T-cell marker, is uncommon in mycosis fungoides.

Predominance of mature helper T cells with the Leu-1+, 2-, 3+, 4+, 5+ phenotype was confirmed in 22 biopsy specimens of mycosis fungoides from 15 patients. Dissection of the T helper/inducer cells into phenotypically distinct subsets was performed with the use of a new monoclonal antibody, anti-Leu-8. One might predict a predominance of Leu-8+ in mycosis fungoides, as the known ratio of Leu-8+/Leu-8- cells is approximately 70/30 in the peripheral blood. Unexpectedly, a deficiency of Leu-8 was demonstrated in 18 of the 22 specimens from 13 of 15 patients. This finding could not be attributed to an artifact of the staining method or to therapy, and was present in early- as well as late-stage disease. Whether neoplastic cells in mycosis fungoides derive from Leu-8-subset of T cells at risk for malignant transformation, or whether there is antigen loss with malignant transformation remains to be determined. Implications of our finding with regard to etiopathogenesis of mycosis fungoides are discussed.

Antibodies, Monoclonal↗

Detection of human T-lymphotropic virus-like particles in cultures of peripheral blood lymphocytes from patients with mycosis fungoides.

Because of seronegativity and absence of a leukemic phase in most patients with mycosis fungoides, a role for the human T-lymphotropic virus type I (HTLV-I) in this disease has remained tenuous. Virus particles are not seen in fresh isolates of skin or blood lymphocytes and the malignant cells (Sézary cells) have been difficult to culture. The availability of growth factors and biomolecular techniques have prompted a renewed attempt to find evidence of virus infection in these patients. We report here the successful culture of blood lymphocytes of 17 patients with mycosis fungoides and 1 patient with the Sézary syndrome. The cells of 2 additional patients failed to grow after 4-6 weeks in vitro. Ultrastructural analysis of the cultures showed an abundance of HTLV-like particles in the specimens of 18 of the 20 patients. Preliminary immunohistochemical studies carried out with various antisera directed against HTLV-I and the polymerase chain reaction utilizing a probe for a conserved region of the pol gene of HTLV-I were positive on only a portion of the specimens. Although definitive characterization of this organism awaits further analysis, it seems likely that circulating lymphocytes of all patients with mycosis fungoides harbor a virus that morphologically resembles HTLV-I.

Blotting, Southern↗

Symptomatic intracranial involvement by mycosis fungoides.

Involvement of the central and peripheral nervous system by mycosis fungoides is rare and usually occurs in the presence of widespread cutaneous and visceral disease. We describe a patient with mycosis fungoides, previously confined to the skin, who developed intracranial parenchymal disease with no evidence of systemic involvement.

Central Nervous System Diseases↗

Mycosis fungoides palmaris et plantaris: successful treatment with the carbon dioxide laser.

Mycosis fungoides palmaris et plantaris (MFPP) is an uncommon form of mycosis fungoides with main involvement on palms and soles. The lesions may be quite hyperkeratotic. A 56-year-old patient with MFPP was successfully treated with the carbon dioxide (CO2) laser. There was no recurrence of disease at treated sites and excellent cosmetic results were obtained. Treatment with the CO2 laser may be considered a therapeutic option.

Foot↗

Granulomatous mycosis fungoides with small intestinal involvement and a fatal outcome.

We report a case of granulomatous mycosis fungoides that progressed into fatal gastrointestinal involvement 4 years after the onset of skin lesions, despite improvement of the skin lesions in response to a combination of PUVA and systemic interferon-gamma therapy. Histological examination showed Pautrier's microabscesses with granuloma annulare-like features and sarcoidal granuloma formation in the plaque stage, proliferation of blast-transformed atypical lymphocytes with persistent granuloma formation in the tumour stage, and metastatic lesions. A literature review of granulomatous mycosis fungoides revealed that 11 of the 24 reported cases died of the disease, and like our case, seven died within 5 years. We suggest that mycosis fungoides with granulomatous reactions does not indicate a favourable prognosis.

Aged↗

Mycosis fungoides of thyroid diagnosed by fine needle aspiration.

Fine needle aspiration of a poorly defined thyroid nodule in a patient with mycosis fungoides revealed convoluted mononuclear cells consistent with involvement by mycosis fungoides. I have briefly reviewed the literature on extracutaneous involvement, and discussed the potential usefulness of fine needle aspiration studies in diagnosing visceral or lymphatic mycosis fungoides.

Biopsy, Needle↗

Intraepidermal but not dermal T lymphocytes are positive for a cell-cycle-associated antigen (Ki-67) in mycosis fungoides.

The Ki-67 antibody, which reacts with nuclei of actively proliferating cells, was used in an immunohistochemical study to determine if there was any difference between T cells located in the epidermis rather than the dermis, in mycosis fungoides. In 12 of 14 cases of patch/plaque stage mycosis fungoides, the epidermal T cells were Ki-67 positive, while the dermal T cells were Ki-67 negative in all cases. Both epidermal and dermal T cells belonged primarily to the memory-versus-naive subset. The intraepidermal Ki-67-positive T cells were slightly larger than the dermal Ki-67-negative cells and could be easily distinguished from occasional basal keratinocytes that were also Ki-67 positive. We conclude that dermal T cells, despite expressing HLA-DR and a memory phenotype, are essentially in a resting (Go or noncycling state) in mycosis fungoides. Furthermore, it appears that the movement of T cells into the epidermal compartment is associated with activation and entry into the cell cycle. Such intraepidermal activation may lead to lymphokine release, and play an important pathophysiologic role in mycosis fungoides.

Antigens, Differentiation↗

Mycosis fungoides involving the brain and optic nerves.

In a patient with long-standing plaque-stage mycosis fungoides, eye pain, a visual field defect, decreased acuity, and a swollen optic disc developed. A lymphomatous infiltrate consistent with mycosis fungoides was found in the brain at craniotomy and in the optic nerves at autopsy. Ten months elapsed between the presenting symptoms and death and during this period the patient's symptoms were controlled with prednisone therapy. The optic nerves as well as the brain can be involved with mycosis fungoides.

Brain↗

Autoimmune anti-T cell antibodies in the sera of patients with mycosis fungoides.

The serum of persons with mycosis fungoides contains IgG antibody believed to be specific for T cells, similar to the auto-antibodies reported in patients with systemic lupus erythematosus, Hodgkin's disease and sarcoidosis. The antibody is present during the active, and disappears during the inactive, phase of the disease.

Adult↗

Bullous reaction to topical methchlorethamine in mycosis fungoides.

We describe a patient with mycosis fungoides who developed a bulla shortly after the start of topical methchlorethamine. Dermal-epidermal separation has been reported to occur with topical methchlorethamine on histological examination. It is plausible that the compromised dermal-epidermal junction may manifest as a clinical bulla after a secondary insult. To our knowledge, clinical bulla formation due to topical methchlorethamine has not been reported.

Aged↗

Adolescence mycosis fungoides: an unusual presentation with hypopigmentation.

Mycosis fungoides (M.F.) is a rare cutaneous malignancy of childhood and adolescence. The disease commonly presents with the classic sequence of erythematous patches and plaques. Hypopigmentation as the presenting symptom is distinctly rare. This is the 9th case report of M.F. presenting with hypopigmentation in a patient under 20 years of age. The disease was only partially controlled with PUVA therapy, necessitating nitrogen mustard treatment.

Adolescent↗