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Immunologic studies documenting the development of mycosis fungoides following successful therapy of a large-cell lymphoma.

We describe a patient who developed mycosis fungoides, a T-cell neoplasm, two years following the chemotherapy and radiotherapy of a diffuse, large-cell ("histiocytic") lymphoma. Immunologic studies documented a "null-cell" phenotype for the large-cell lymphoma. Therefore, the proliferation of two distinct malignant lymphoid clones is believed to have developed in the patient. The value of immunologic marker studies in this case was to aid in the diagnosis of the two separate neoplasms, as well as to aid in the selection of appropriate specific therapy directed against each neoplasm. As the association of large-cell lymphoma and mycosis fungoides in one patient represents a very rare event, we discuss several hypotheses about the possible interrelation of the two neoplasms.

Adult↗

[Mycosis fungoides bullosa--a case report (author's transl)].

An extremely rare variant of mycosis fungoides, presenting with bullous lesions, is reported. Histopathological examination of the skin revealed subepidermal blister formation, a diffuse mononuclear infiltrate in the uppermost parts of the dermis and the characteristic intraepithelial Pautrier microabscesses. Lutzner cells were found at the ultrastructural level. The mechanism of blister formation in mycosis fungoides is outlined.

Aged↗

Topical nitrogen mustard in mycosis fungoides.

A twelve-year experience in thirty-three patients suffering from early mycosis fungoides in the plaque stage confirmed the effectiveness of topical nitrogen mustard (chlormethine) therapy. Fourteen patients were in complete remission at the latest time of observation and seven 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. Another 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 and no haematological side-effects were observed among the patients. The treatment as a whole was well tolerated.

Administration, Topical↗

The role of total skin irradiation with electrons in the management of mycosis fungoides.

The study presents a group of 176 patients with biopsy proven mycosis fungoides treated with total skin irradiation with electrons. The treatment resulted in a complete disappearance of all cutaneous lesions in 117/176 (66 percent) of the patients. The rate of complete remission was dose-dependent and was inversely dependent on the extent of the disease. Nineteen patients have been continuously free of any evidence of mycosis fungoides 4-14 years following a single course of treatment and may be considered as cured. Most of the patients presenting with significant peripheral lymphadenopathy (Stage II) eventually relapsed with cutaneous lesions and 37 percent of these patients developed extra-cutaneous dissemination. The prognosis of patients with extracutaneous involvement was poor and no patients in this series survived for more than three years after the histological diagnosis of extractaneous dissemination. The role of total skin irradiation with electrons in the management of each stage of the disease, given either alone or in combination with other treatment modalities, is discussed.

Electrons↗

Exposure to chemicals, physical agents, and biologic agents in mycosis fungoides and the Sézary syndrome.

The history of occupational, environmental, and/or iatrogenic exposure to potential carcinogenic agents was obtained at the time of onset of skin disease in 43 of 44 patients with cutaneous T-cell lymphoma (mycosis fungoides and the Sézary syndrome) entering a National Cancer Institute therapeutic trial. A history of multiple exposures to these agents was common, the two most frequent being chemicals (91% of patients) and drugs (86%). Mean duration of expsosure was 13 years for chemicals and 18 years for drugs. The most common chemicals were air pollutants (39%), pesticides (36%), solvents and vapors (30%), and detergents and disinfectants (14%). Increased severity of disease was seen with increaed duration of chemical exposure in stage V cutaneous T-cell lymphomas only. The most frequent drugs besides tobacco (86%) were analgesics (20%), tranquilizers (18%), and thiazides (14%). Second cancers occurred in four patients, including two renal cell carcinomas, and a family history of cancer was present in 11. Fourteen of 22 patients questioned had recurrent herpes simplex. Patients with chronic skin disease who have long-term exposure to combinations of chemicals, physical agents, and biologic agents, are heavy smokers, or have recurrent herpes simplex appear to be prime candidates for developing mycosis fungoides or the Sézary syndrome.

Adult↗

Subpopulations of T lymphocytes in a patient with fulminant mycosis fungoides.

A patient with fulminant Mycosis fungoides was found to have a very high percentage of T lymphocytes with Fc receptors for IgG and a low percentage of T lymphocytes with Fc receptors for IgM. The total number of T lymphocytes was normal, but the in vitro function of the lymphocytes was depressed in short-term cultures, though not in cultures of 5 days' duration. T lymphocytes with Fc receptors for IgG are considered to have suppressor functions, and the immunological changes in this patient may be explained by an increased suppressor cell activity. Other investigators have proposed that the abnormal lymphocytes in Mycosis fungoides are T helper cells. After treatment with electron beam and transfer factor, our patient developed uraemia due to uric acid crystallization in the kidneys. This complication to the treatment given seems not to have been reported before.

Aged↗

Elevated colchicine sensitivity of dermal lymphocytes in mycosis fungoides.

Lymphocytes in chronic lymphocytic leukaemia and in other malignant lymphomas exhibit an extreme sensitivity to colchicine and are cytolysed in vitro by 10(-6) M colchicine. This characteristic can be quantified with the colchicine sensitivity index which is used for diagnostic purposes. We have demonstrated that lymphocytes liberated from skin infiltrates in mycosis fungoides (6 patients) show an elevated colchicine sensitivity of 44 (p < 0.01); in contrast, the index of lymphocytes isolated from dermal infiltrates of reactive inflammatory dermatoses (9 patients) is low, being only 23. Determination of the colchicine sensitivity index can be helpful in the diagnosis of mycosis fungoides.

Adult↗

Focal contact sensitivity to nitrogen mustard in lesions of cutaneous T-cell lymphoma (mycosis fungoides).

A report is presented on a patient with mycosis fungoides who developed a remarkable focal contact sensitivity to topically applied nitrogen mustard in aqueous solution (1/5000). Although the nitrogen mustard was applied over the entire skin surface, repeated challenge demonstrated that the eczematous contact dermatitis reaction was limited precisely to the areas of clinically identifiable mycosis fungoides. The surrounding normal skin showed no response. The basis for the surprising focal induction and localization of contact dermatitis is not known but might reflect the presence of a clone of malignant T-lymphocytes which became specifically sensitized to the nitrogen mustard.

Administration, Topical↗

Transfer factor therapy in mycosis fungoides: a double-blind study.

Sixteen patients with mycosis fungoides (MF) were given either active transfer factor (TF) or heat-inactivated TF as additional therapy to topical nitrogen mustard or PUVA. The TF was prepared from non-selected healthy blood donors. The clinical evaluation after 2 years of therapy showed that among 8 patients treated with active TF, none went into complete remission of their disease 4 patients had partial remission, one was unchanged, 2 progressed, and one died of active MF. In the placebo-treated group, 5 patients achieved complete remission and 2 partial remission. One patient died early in the trial due to cardiac disease. Immunological studies during the first year of therapy revealed cutaneous anergy towards tuberculin in most patients. This anergy did not change during TF therapy and differed from normal lymphocyte reactivity in vitro after tuberculin stimulation. At the start of treatment the patients had diminished levels of T lymphocytes in peripheral blood. A temporary increase was observed in the total number of T lymphocytes in patients after one month of treatment with active TF. After one year the T lymphopenia had disappeared in both groups. The mitogen reactivity of lymphocytes was found to be normal (PHA, PWM) or somewhat reduced (Con A). It is concluded that under the conditions employed in this trial, TF was not able to prevent progression of early mycosis fungoides, when viewed over a period of 2 years.

Administration, Topical↗

Oral retinoid in combination with bleomycin, cyclophosphamide, prednisone and transfer factor in mycosis fungoides.

Oral retinoids seem to have been of great benefit in a non-randomized study on advanced mycosis fungoides using two different chemotherapy regimens, one with retinoid, the other without. Both groups also received a 3-drug chemotherapy with bleomycin, cyclophosphamide and prednisone. Complete remission including all signs of lymph-node involvement was found in 8 of 10 patients of the retinoid treated group, while none went into complete remission in the control group. All in the control group died between 3 and 12 months after therapy, whereas all but one in the retinoid treated group are alive. Other treatment differences between the groups were related to the use of transfer factor, topical treatment, and steroid administration. These differences make a final evaluation of the use of retinoids in mycosis fungoides difficult at the present stage. Further studies are needed.

Administration, Oral↗

Langerhans cells and their precursors with S100 protein in mycosis fungoides.

Twenty-four cases of mycosis fungoides and two cases of Sézary's syndrome were studied with special reference to the infiltration and proliferation of Langerhans cells stained with anti-S100 protein antibody. The number of S100+ histiocytes (Langerhans cells and their precursors) ranged from 104 to 345 (average 188) per 1,000 lymphoid cells in the erythematous and plaque stages, while it decreased to 4-71 (average 34) per 1,000 lymphoid cells in the tumor stage. Conversely, the number of lymphoid cells averaged 6 and 10 per S100+ histiocyte in the erythema and plaque stages, respectively, while it increased to 21.0 per S100+ histiocyte in the tumor stage. The mitotic index of lymphoid cells correlated with the lymphocyte/S100+ histiocyte ratio. A small number of S100+ histiocytes in mycosis fungoides indicated poor prognosis.

Adult↗

[Disseminated type of pagetoid reticulosis? Mycosis fungoides?].

We present a case of a 69 year old man who died with a progressive systemic disease. This case demonstrated a multiple clinical evidence with aspects of psoriasis, parapsoriasis en plaques, mycosis fungoides, lymphogranulomatosis and finally of the disseminated type of pagetoid reticulosis. The last biopsies always demonstrated the typical histology of pagetoid reticulosis. The earlier biopsies revealed findings similar to mycosis fungoides and even in part similar to lymphogranulomatosis.

Aged↗

Selective immunological evaluation of mycosis fungoides.

Tests of specific aspects of immune function were conducted on 15 patients with clinically and histologically evident mycosis fungoides of various stages. These tests included peripheral blood lymphocyte counts, T and B cell counts, mitogen stimulation tests to phytohemagglutinin (PHA), concanavalin A (con A), and pokeweed mitogen, as well as determinations of IgE levels and antinuclear factor (ANF). We found that mycosis fungoides patients, as a group, had normal peripheral blood lymphocyte, T cell, and B cell counts, but had decreased stimulation to PHA and con A. When evaluated by stage of disease, the peripheral blood lymphocytes, T and B cell counts did not differ significantly from normal, at least until stage V. Concanavalin A stimulation was decreased by stage II disease, and PHA was decreased by stage III, indicating at least a selective immunological deficit in the plaque stage or in later disease. Values of IgE and ANF were rarely abnormal.

Adult↗

Nontraditional treatment of mycosis fungoides in a dog.

A 14-year-old Dachshund with plaque-stage mycosis fungoides was treated and kept free of lesions for 25 months, using combination drug therapy of a placental lysate and low-dose prednisone. Mycosis fungoides has a poor prognosis and has had a history of poor response to treatment. Placental lysate with low-dose prednisone may offer an alternate therapeutic regimen in this disease.

Animals↗

Localized mycosis fungoides not manifesting as Woringer-Kolopp disease.

A 68-year-old man had a solitary, eczematiform lesion of two years' duration on the digital dorsal surface of his right foot. Despite its innocuous appearance, the lesion showed histologic characteristics of advanced, plaque-type mycosis fungoides. The patient's clinically benign yet histologically malignant disease manifestation is discussed. The distinction between localized mycosis fungoides and Woringer-Kolopp disease is also reviewed.

Aged↗

Sarcoidosis associated with mycosis fungoides.

In November 1966, a 47-year-old black woman had a skin eruption and an abnormal chest roentgenogram that were found to be due to sarcoidosis. In September 1974, nodular lesions and subsequently generalized exfoliative dermatitis developed that were found to be due to mycosis fungoides. The concurrence of sarcoidosis and mycosis fungoides has been recorded only once in the literature. The depression of cell-mediated immunity found in this patient and other sarcoid patients suggests that impairment of the host's immunosurveillance system may predispose to the development of T-cell lymphomas.

Female↗

[Mycosis fungoides with central nervous system involvement].

Mycosis fungoides at present is classified as a malignant cutaneous lymphoma of T-cell-type. Involvement of every organ is generally possible. Referring to autopsies involvement of the central nervous system is found by chance in about 10%. Since the first description of Mycosis fungoides there are only 20 reports published dealing with a clinical picture due to involvement of the central nervous system. Based on a survey of literature the scale of individual reports concerning involvement of the nervous system is analysed and a report is given on a patient with such a malady confirmed by autopsy.

Adult↗

[Whole-skin electron therapy: a therapeutic concept for mycosis fungoides and Sézary syndrome. 2: Therapy].

The alterations of mycosis fungoides of a malignant T-cell lymphoma are extremely radiosensitive. In the treatment of the cutaneous manifestation of mycosis fungoides, the whole-skin electron therapy is superior to the superficial application of mechlorethamine (NH2), to photochemotherapy with psoralen and ultraviolet radiation as well as to chemotherapy as monotherapy and combined therapy. Complete remissions are to be expected in 93% of all cases after application of a high total dose of more than 30 Gy. After less than 10 Gy, this rate is 18%. A combination of whole-skin electron therapy (30 Gy) with chemotherapy or with a total lymphoid irradiation with 30 Gy represents a new approach for the treatment of advanced tumor stages.

Adult↗