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Immunotactoid glomerulopathy associated with mycosis fungoides.

A patient with mycosis fungoides developed a nephrotic syndrome. Renal biopsy revealed deposits of a highly organized fibrillar material which did not stain with the typical amyloid stains; this picture was consistent with the diagnosis of non-amyloidotic fibrillary glomerulopathy or immunotactoid glomerulopathy. We believe this is the first case reported of immunotactoid glomerulopathy associated with mycosis fungoides. Possible pathogenetic implications are discussed with reference to previous publications.

Female↗

Mycosis fungoides with rapidly progressive pulmonary infiltration.

A case of mycosis fungoides is reported in which diffuse pulmonary infiltration was noted in six days. Although pulmonary infiltration with the abnormal Sézary cells is a frequent occurrence in the extracutaneous spread of mycosis fungoides, this is the first case report documenting the rapidity of spread.

Aged↗

Mycosis fungoides: manifestations on computed tomography.

Mycosis fungoides is a malignant lymphoma of the skin that causes intense erythema, plaques, edema, and induration. The primary computed tomographic findings of mycosis fungoides, i.e., skin thickening, tumorous infiltration, edema of the soft tissues, and lymphadenopathy, are presented in two patients.

Edema↗

Cytotoxic mycosis fungoides evolving from pityriasis lichenoides chronica in a seventeen-year-old girl. Report of a case.

Pityriasis lichenoides chronica and its acute form, pityriasis lichenoides et varioliformis acuta, are skin diseases of unknown origin, that probably represent a hypersensitivity reaction to an infective agent. Pityriasis lichenoides is often a benign disorder but, because of the presence of a clonal T cell population detected both in the chronic and acute forms, some authors have suggested that it may belong to the group of primary cutaneous T cell lymphomas. Although various studies have clearly documented no significant association between pityriasis lichenoides and malignant lymphomas, cases of long-standing pityriasis lichenoides evolving into mycosis fungoides have been described. Herein we report the case of a girl suffering from pityriasis lichenoides since the age of 11 years, subsequently developing a CD45RO+, CD8+, TIA-1+ mycosis fungoides.

Adolescent↗

Secondary pancreatic involvement of mycosis fungoides detected by a clinically palpable mass.

A patient with a 4-year history of mycosis fungoides, who presented a pancreatic spreading of the disease, detected by a clinically palpable mass, is reported and discussed with a review of the literature. Mycosis fungoides (MF) is a T cell lymphoma primarily localized to the skin. Laparoscopic staging and autopsy studies, however, have shown that lymph node involvement or visceral spreading is common in the course of the disease [1-3]. Among visceral manifestations of MF, pancreas infiltration is found in 20-40% of autopsied cases [1, 2, 4]. Occurrence of a palpable pancreatic mass has never been described. We report a patient with a long-standing MF, and a secondary pancreatic extension detected by a palpable mass.

Aged↗

Mycosis fungoides with monoclonal gammopathy, hypereosinophilia, and hyper-IgE.

A 61-year-old female with a well-established Mycosis fungoides also had a marked hypereosinophilia, a monoclonal gammopathy IgGk type, and an elevated IgE level. Such unusual association of abnormalities of the immune system in a given Mycosis fungoides patient has not been described before in the absence of any other underlying cause.

Eosinophils↗

A study of the photosensitive factor in relation to skin lesions of mycosis fungoides patients.

Some mycosis fungoides patients show abnormal light reactions in apparently normal skin. Clinically it is observed that lesional skin most easily burned during photochemotherapy. For this reason phototests to broad wavelength regions were performed in 9 mycosis fungoides patients, close to skin lesions and at 3 and 10 cm from cutaneous lesions. The light doses giving the minimal erythemal dose, were of about the same magnitude at a distance of 3 and 10 cm from the lesions. One third of the patients showed abnormal reactions following UVA and UVB. More abnormal reactions were seen close to lesional skin, and a displacement towards increased light sensitivity was demonstrated.

Aged↗

Transfer factor in mycosis fungoides: three years experience.

13 patients with clinically and histologically verified mycosis fungoides were treated with transfer factor as additional therapy to the hitherto conventional treatment after this had failed. After approximately 3 years, complete remission was present in 3 patients, 4 patients were significantly improved and registered as being in partial remission, while the condition was registered as no change in 3 patients. 1 patient was found worse, 1 patient had died after discontinuation of therapy and 1 patient was out of the study. In this case treatment was withdrawn because of the development of contact urticaria to nitrogen mustard, her basic therapy. The number of T lymphocytes, which was low prior to treatment, increased to normal values during the therapeutic period. During the first year a decrease in serum IgE was noted. The results of the clinical evaluation seem to indicate that transfer factor may be of value as an additional therapeutic agent in mycosis fungoides. Controlled investigations are needed and are in progress.

Adolescent↗

The Sézary cell in the blood of patients with mycosis fungoides.

A study of 96 patients with an original diagnosis of mycosis fungoides revealed that 84 did not have circulating Sézary cells. Of the 12 patients with Sézary cells, only 3 had more than 15%. The 12 patients had benign and malignant skin diseases. The Sézary cell does not seem to have any importance in the pathogenesis of mycosis fungoides.

Adolescent↗

Total skin electron irradiation in mycosis fungoides.

Total skin irradiation with fast electrons (4 MeV, 3,500 rad) was applied in 10 cases of histologically proven mycosis fungoides without any signs of internal dissemination. The technique is described. Complete disappearance of both lesions and symptoms occurred in all patients after treatment. However, half of the patients relapsed after the initial irradiation and were subsequently treated with a booster dose of 1,000 rad 4 MeV electrons or with topical nitrogen mustard. Irradiation with fast electrons seems to be of great importance in the treatment of mycosis fungoides.

Female↗

Distribution of HLA antigens in patients with mycosis fungoides.

19 patients with plaque-style mycosis fungoides have undergone determination of HLA antigen distribution. Deviations from the expected distribution frequency occurred with the antigens of the W19 group comprising A29, W30, W31 and W32 and of B8 and A1. The W19 group deviation was statistically significant (chi2 = 9.7 p value less than 0.005). The significance of this observation in relation to reported immunological abnormalities in mycosis fungoides is discussed.

Adult↗

Hodgkin's disease and mycosis fungoides in a married couple.

Hodgkin's disease and mycosis fungoides are malignancies in which there is abnormal T lymphocyte proliferation. The authors report the occurrence of Hodgkin's disease and mycosis fungoides in a husband and wife, respectively. This represents another instance of clustering of cases of T lymphocyte malignancies.

Adult↗

Laryngeal involvement by mycosis fungoides.

Symptomatic involvement of the larynx by mycosis fungoides is rare. We report a case of mycosis fungoides occurring in the larynx of a 74-year-old man. The laryngeal localization was the first visceral manifestation of the disease to be detected clinically.

Aged↗

Mycosis fungoides: subcutaneous and visceral tumors, orbital involvement, and ophthalmoplegia.

A patient with advanced severe mycosis fungoides presented several unusual features, including prominent lesions of the palate and tongue and an orbital tumor with exophthalmos and ophthalmoplegia. A hitherto undescribed feature was the development of multiple, massive subcutaneous tumors unrelated to the dermis or to lymph nodes, and large tumors in the connective tissues of the buttock, flank, and retroperitoneum. The usual sites of extracutaneous dissemination of mycosis fungoides--lymph nodes, spleen, liver, lungs, and blood--were not demonstrably involved. This may be a new pattern of dissemination for this disease. Of practical importance is the immediate and complete relief of exophthalmos and ophthalmoplegia that was obtained with emergency radiotherapy.

Aged↗

Mycosis fungoides involving the central nervous system.

The clinical records of nine patients, followed at the University of Chicago, with mycosis fungoides (MF) and neurologic deficits due to direct CNS involvement were reviewed. Lymph node and visceral involvement was present in all patients. Clinical findings included cranial nerve deficits in four patients, altered mental status in eight, gait disturbances in three, and leg weakness in one. Helpful diagnostic studies included lumbar puncture with CSF cytology, computerized tomography (CT) brain scans, brain biopsy, and myelogram. Two patients, who were treated with radiation therapy (RT), survived 14 and 40 weeks, respectively. Survival of less than 4 weeks limited treatment in the other patients. Autopsies were performed on six patients and revealed meningeal infiltration and perivascular infiltration of the brain. In addition, the cerebral parenchyma was infiltrated in one patient and a lymphomatous tumor was found in the brainstem of another. We conclude that the varied clinical and pathologic features of CNS involvement with mycosis fungoides resemble those seen in leukemias and other lymphoproliferative disorders. Better awareness of the clinical features may permit earlier diagnosis and initiation of therapy.

Adult↗

Thyrotoxicosis after denileukin diftitox therapy in patients with mycosis fungoides.

CONTEXT: Denileukin diftitox is a recombinant novel fusion protein of diphtheria toxin and the ligand-binding domain of human IL-2. Denileukin diftitox binds to the high-affinity IL-2 receptor on the cell surface, and it is internalized by endocytosis and enzymatically cleaved. The cytotoxic A-fragment of the toxin inhibits protein synthesis and causes cell death. OBJECTIVE: The objective of this study was to recognize thyrotoxicosis in association with denileukin diftitox therapy. DESIGN: This study was a retrospective case series. SETTING: The setting of this study was a comprehensive cancer center. PATIENTS: Eight mycosis fungoides patients who were receiving 9 or 18 microg/kg.d iv denileukin diftitox for 5 d every 3 wk were identified with thyrotoxicosis. INTERVENTION(S): Thyroid testing was performed. Hypothyroidism after thyrotoxicosis was treated. RESULTS: In eight mycosis fungoides patients who developed transient thyrotoxicosis during therapy, thyroid function tests were normal before onset of therapy. Clinical thyrotoxicosis developed within days of the first cycle of denileukin diftitox therapy in four patients and after the second cycle in the other four patients. Symptoms included tremors, nervousness, tachycardia, diarrhea, and weight loss. After cessation of denileukin diftitox, thyrotoxicosis resolved in all patients; two became euthyroid, and five became hypothyroid, requiring levothyroxine therapy. One patient was lost to follow-up. CONCLUSIONS: Monitoring thyroid function before and during treatment with denileukin diftitox is recommended. Symptomatic thyrotoxicosis may be missed due to other acute reactions to the drug, and subsequent hypothyroidism may develop.

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↗

Mycosis fungoides/Sézary syndrome: a report of three cases treated with Campath-1H as salvage treatment.

We report the use of Alemtuzumab (Campath-1H) as salvage treatment in three patients with advanced mycosis fungoides/Sézary syndrome who had previously been treated with conventional chemotherapy. Two patients (case 1 and case 2), aged 42 and 68 yr, respectively, were heavily pretreated (more than three prior therapy regimens, including autologous transplant in case 2) and refractory to conventional chemotherapy, and the third patient (case 3), aged 80 yr, who had refused any chemotherapy, had been resistant to treatment with cyclosporine and steroids. Campath-1H was administered intravenously, after an escalating dose from 3 to 10 mg, at the dose of 30 mg, three times weekly, to a total dose of 1080, 223, and 480 mg, respectively. The patients with Sézary syndrome (case 2 and case 3) showed clearance of circulating Sézary cells and clinical improvement of the skin lesions after 2 wk of treatment. Two patients (case 1 and case 3) completed the treatment (12 and 6 wk) without significant toxicity, the former achieving a partial response and the latter a clinical complete response. The patient (case 2), who suffered from ischemic cardiopathy and diabetes, quickly achieved a clinical improvement of the Sézary syndrome, but he died because of a myocardial infarction after 3 wk of treatment. Our report shows that the treatment with Campath-1H is active even in patients with advanced refractory mycosis fungoides/Sézary syndrome. Further clinical observations on a larger cohort of patients are needed to establish if Campath-1H may have a role as first line therapy in addition to conventional therapy including chemotherapy.

Adult↗