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PubMedDate not supplied

Mycosis fungoides with pulmonary involvement. Cytopathologic findings.

In order to define the cytologic features of pulmonary involvement by mycosis fungoides, 15 respiratory cytology specimens from four patients with biopsy-proven pulmonary mycosis fungoides were reviewed. The presence in sputum smears of occasional small or large cerebriform mononucleated cells against a background of numerous atypical lymphocytic cells permitted an antemortem cytologic diagnosis of probable or definite dissemination of mycosis fungoides with pulmonary involvement. Similar cells were seen in aspiration smears. The lymphocytic infiltrates were similar to those in corresponding skin biopsies in each case. The distinctive cytologic findings in these cases may therefore help to determine the underlying etiology of pulmonary lesions and may contribute to the antemortem diagnosis of visceral dissemination of mycosis fungoides.

Biopsy, Needle↗

PubMed1996

Mycosis fungoides presenting as an acquired ichthyosis.

A 25-year-old patient with an acquired ichthyosis-like condition is described. Histologic and molecular biologic examination of the fine, whitish, scaling skin lesions suggested the diagnosis of mycosis fungoides. Typical lesions of mycosis fungoides, such as patches, plaques, or tumors, were not present. To the best of our knowledge, this is the first case of mycosis fungoides presenting solely with the clinical features of acquired ichthyosis. The sudden appearance of ichthyosiform skin changes in adults must be taken seriously and the underlying cause must be evaluated. A skin biopsy should be performed in all cases of acquired ichthyosis to rule out mycosis fungoides.

Adult↗

PubMed1991

PUVA-induced lymphomatoid papulosis in a patient with mycosis fungoides.

The occurrence of lymphomatoid papulosis in patients with cutaneous lymphoma, particularly mycosis fungoides, has been described in medical literature. A 68-year-old woman affected by mycosis fungoides in the plaque stage noticed that multiple papulonodular lesions of lymphomatoid papulosis developed suddenly after a few sessions of PUVA therapy. The PUVA induction of lymphomatoid papulosis was confirmed by the appearance of new lesions after a second cycle of PUVA exposure on a limited area of the body. Complete regression of all PUVA-induced lymphomatoid papulosis lesions was achieved within a few weeks with oral prednisone and topical steroids. During the entire treatment the patches and plaques of mycosis fungoides persisted unchanged.

Aged↗

PubMed2005

Mycosis fungoides--a retrospective study of 40 cases in Hong Kong.

BACKGROUND: Mycosis fungoides is rare in Hong Kong and oriental data on the disease are lacking. METHODS: This is a multiclinic, 13-year, retrospective study to determine the clinicopathologic characteristics, treatment, and disease outcomes of 40 patients with mycosis fungoides/Sézary syndrome seen in the Social Hygiene Service, Hong Kong. RESULTS: There were 27 males and 13 females with a mean age at diagnosis of 56.4 years. Based on figures in the Social Hygiene Service alone, the incidence in Hong Kong was estimated to be 0.044 per 100,000. Eighty-five per cent of patients presented with skin-limited disease, and pruritus was absent in 40% of patients. A mean of 1.48 biopsies was needed to establish the diagnosis, and only 58% of the mycosis fungoides skin biopsies were reported to be histologically diagnostic of the disease. Atypical lymphocytes, epidermotropism, interface changes, and Pautrier's microabscesses were the four most frequently encountered features that attained statistical significance. Sixty-nine per cent of patients were treated with psoralen-UVA as their initial therapy, and the complete response and relapse rates were 78.3% and 66.6%, respectively. Disease progression to more advanced stages was only seen in 15% of patients. The 5-year survival rates for the whole group and for stage IA and IB patients were 88.8% and 100%, respectively. CONCLUSION: Mycosis fungoides is rare amongst Hong Kong Chinese and the majority present with skin-limited nonprogressive disease.

Aged↗

PubMed1975

Immune response to primary immunization with brucella antigen in mycosis fungoides.

The immune response to primary immunization with brucella antigen was studied in 8 patients with mycosis fungoides and 11 controls. The development of as well cellular as humoral hypersensitivity to brucella antigen was followed by in vitro techniques. Both, patients with mycosis fungoides and controls were studied for previously acquired delayed hypersensitivity to tuberculin by skin test. The distribution of B and T cells was encountered among the patients. Previously acquired cellular immunity was found equal in the two groups, however, induction of cellular hypersensitivity to brucella antigen was found depressed in mycosis fungoides patients compared with the controls. Also the humoral response to brucella antigen could seem depressed, only one of seven patients with mycosis fungoides had a positive titer, while seven of eleven controls had positive titres following vaccination; but the difference between the two groups was not significant.

Adult↗

PubMed1996

PUVA in early mycosis fungoides may give long-term remission and delay extracutaneous spread.

We report a follow-up for 3-18 years of 24 patients with the non-infiltrated patch and infiltrated plaque stage of mycosis fungoides, treated with psoralen photochemotherapy (PUVA) and on resistant infiltration or development of tumours also with fractionated radiation therapy. All patients with patch and limited plaques showed complete remission after initial PUVA therapy. Half of the patients with patch stage mycosis fungoides and also half of the patients with plaque stage mycosis fungoides were in complete remission when the study ended. Most of them had remission periods for years after early PUVA treatment. Patients with more advanced mycosis fungoides needed repeated periods of PUVA therapy. Two patients with extensive infiltrated plaques did not reach complete remission at all during the study but progressed and finally died of their T-cell lymphoma. Another 4 patients with extensive plaque stage mycosis fungoides died after initial complete remission for a maximum of 3 years. In this investigation the clinical evaluation was made by one dermatologist and the histopathological evaluation by one pathologist. This is of importance since in the early stages of mycosis fungoides the diagnosis is challenging and may require a combination of clinical, histopathological and molecular evaluations. The identification of early disease is crucial for the rapid implementation of adequate treatment. The study shows that early PUVA therapy may delay extracutaneous spread and possibly also in some cases be curative.

Adult↗

PubMed1990

[The applied significance of a study of the biochemiluminescence of the blood serum in patients with mycosis fungoides].

Analysis of chemiluminescence levels of blood sera from 64 patients with mycosis fungoides and 44 ones with nontumor dermatoses (eczema, psoriasis, secondary erythroderma) has helped specify some aspects in the pathogenesis of mycosis fungoides. Basing on the findings of this analysis, the authors have developed an auxiliary method for differential diagnosis of mycosis fungoides and nontumor dermatoses, whose clinical patterns are similar, and a method for assessing the tumor cell sensitivity to prescribed polychemotherapeutic complex and finding the optimal period between the courses of therapy.

Adult↗

PubMed1968

Mycosis fungoides. Topical use of nitrogen mustard in recurrent cases.

The management of the patient with mycosis fungoides requires a variety of therapeutic modalities depending on the stage of the disease. Topically applied nitrogen mustard in the early stages of the disease has a beneficital palliative effect. The effects of nitrogen mustard paintings in the later course of the disease have not been previously reported. In the present study, topically applied nitrogen mustard solution was used to control recurrences of mycosis fungoides following electron beam therapy in 11 patients. Each patient received whole body applications of freshly prepared 10 mg per 50 ml solution of mechlorethamine hydrochloride (a nitrogen mustard) in water daily for seven days. In all patients pruritus disappeared within the first week and ulcers and plaques improved or disappeared in two to four weeks. The seven-day courses of mechlorethamine paintings were repeated as recurrences were noted. Mycosis fungoides was controlled by this therapy for periods ranging up to 15 months. Absence of systemic toxicity, a low incidence of cutaneous irritation and application of the treatments at home make topical nitrogen mustard a useful adjunct in the management of the late stages of mycosis fungoides.

Adult↗

PubMed1996

Hodgkin's disease following mycosis fungoides: phenotypic and molecular evidence for different tumour cell clones.

AIMS: (1) To assess the clonality of tumour cells in two patients with mycosis fungoides who subsequently developed Hodgkin's disease; and (2) to determine whether there is a clonal relation between these two disorders. METHODS: Cutaneous tissue samples involved by mycosis fungoides and lymph nodes involved by Hodgkin's disease from both patients were investigated by immunohistochemistry and the polymerase chain reaction. RESULTS: Mycosis fungoides tumour cells in both patients expressed multiple T cell associated antigens; Reed-Sternberg (RS) cells had the null phenotype. T cell receptor gamma chain genes were clonally rearranged in mycosis fungoides cells but not in RS cells, including variants, in both patients. In the patient with intermediate transformation to large cell lymphoma, immunoglobulin heavy chain genes were rearranged in the cutaneous tumour, but not in the lymph node involved by Hodgkin's disease. CONCLUSION: The divergent antigen expression and gene rearrangements observed in these two patients strongly suggest that Hodgkin's disease and mycosis fungoides are not derived from a single tumour cell clone.

Clone Cells↗

PubMed2003

Long-term control of mycosis fungoides of the hands with topical bexarotene.

BACKGROUND: Limited Stage IA mycosis fungoides (MF) is often treated with topical steroids, which can cause atrophy, or with nitrogen mustard, which imposes several limitations on the patient's lifestyle. Topical bexarotene is a novel synthetic rexinoid with few side-effects that has shown efficacy for treatment of mycosis fungoides skin lesions in recent Phase II-III clinical trials. The Phase I-II trial involving 67 stage IA-IIA MF patients demonstrated complete response (CR) in 21% and partial response (PR) in 42% of the patients. The median time to response was approximately 20 weeks. In the phase III trial of refractory stage IA, IB and IIA MF, the patients demonstrated a 44% response rate (8% CR). Patients with no prior therapy for mycosis fungoides responded at a higher rate (75%) than those with prior topical therapies. METHODS: Case report of a patient with MF limited to the hands treated with topical bexarotene 0.1% gel in a open label phase II clinical trial. RESULTS: Partial response occurred after 2 weeks of topical bexarotene therapy and the lesions were well controlled for 5 years using bexarotene monotherapy, with only occasional mild local irritation. CONCLUSIONS: Topical bexarotene is effective as long-term treatment monotherapy for limited MF lesions. To our knowledge this is the longest use of the drug by any individual.

Administration, Topical↗

PubMed2004

Follicular mucinosis associated with mycosis fungoides.

A 62-year-old man with a 13-year history of mycosis fungoides presented with a 2-month history of alopecia of the scalp. The mycosis fungoides had remained untreated for the previous 3.5 years. A biopsy specimen from the scalp showed follicular mucinosis in association with mycosis fungoides.

Humans↗

PubMed2003

[Mycosis fungoides in childhood and adolescence with clonal T-cell receptor gamma gene rearrangement. Two cases].

Mycosis fungoides (MF) is a cutaneous T-cell lymphoma (CTCL) characterized by its typical progress in three stages: the patch-, the plaque- and the tumour-stage. The incidence of mycosis fungoides rises with age and the average age at presentation is about 50. Children and adolescents are rarely affected and there are only few reports in the literature. We report a 12- and a 15-year-old boy showing refractory skin lesions not typical for mycosis fungoides. The histo- and immunohistological investigations and the detection of clonal T-cell receptor gamma gene rearrangements confirmed the diagnosis of early onset mycosis fungoides in both cases.

Adolescent↗

PubMed1995

Prognostic factors in erythrodermic mycosis fungoides and the Sézary syndrome.

BACKGROUND AND DESIGN: There are no large studies evaluating patients with erythrodermic mycosis fungoides and Sézary syndrome to determine the important prognostic factors that may influence survival. This is important since new treatment modalities have been proposed as superior to existing primary therapies. We performed a retrospective cohort study of 106 patients with erythrodermic mycosis fungoides and Sézary syndrome, followed up in the Stanford (Calif) Mycosis Fungoides Clinic, to define the important prognostic factors in this group. RESULTS: Patients younger than 65 years have a more favorable survival profile than those 65 years or older (P < .005). Longer duration of symptoms before diagnosis ( > or = 10 years) tends to be associated with more favorable prognosis (p = .055). Lymph node stage is significantly correlated with survival; patients with overall stage III disease have more favorable prognosis than those with stage IV disease (P < .001). Patients with circulating Sézary cells in their blood have a significantly worse prognosis than those without (P < .005). Patient sex or race had no significant effect on overall survival outcome. Three distinct prognostic groups were identified, "favorable," "intermediate," and "unfavorable," according to the number of unfavorable prognostic factors (P < .005). The median survival in each group is 10.2, 3.7, and 1.5 years, respectively. CONCLUSIONS: In patients with erythrodermic mycosis fungoides and Sézary syndrome, the important prognostic factors are patient age at presentation, the overall stage, and peripheral blood involvement. Survival varies widely, depending on these variables. These prognostic factors should be evaluated when analyzing survival and/or treatment efficacy data of these patients.

Adult↗

PubMed1984

Combination chemotherapy for mycosis fungoides with cyclophosphamide, vincristine, methotrexate, and prednisone.

Seven patients with stage IV mycosis fungoides [TNM classification] have been treated with combination chemotherapy consisting of cyclophosphamide, vincristine, methotrexate, and prednisone [COMP]. A complete response rate of 57% was produced with an overall response of 100% in patients having limited prior steroid and/or chemotherapy. Complete responses range from 4+ to 20 months [median, 11 months]. Overall survival ranges from 2 to 40+ months [median, 12+ months]. The protocol was well tolerated except for one treatment-related death. Combination chemotherapy can produce effective remissions in patients with advanced mycosis fungoides. TNM classification should be considered in staging patients with mycosis fungoides for comparison of different treatment regimens.

Adult↗

PubMed2003

A complete and durable response to denileukin diftitox in a patient with mycosis fungoides.

We describe a patient with stage IA mycosis fungoides treated with denileukin diftitox as part of a randomized phase III trial. The patient had a rapid and complete response and has remained in remission for more than 5 years without maintenance therapy. Although denileukin diftitox is not usually considered for patients with early-stage mycosis fungoides, our experience provides anecdotal evidence that fusion toxin therapy may be worth considering as a treatment for patients with stage I disease.

Aged↗

PubMedDate not supplied

Granulomatous mycosis fungoides.

We report a new observation of granulomatous mycosis fungoides. The diagnosis was able to be made only after performing multiple biopsies during the course of the disease. Initial evolution was rapidly favourable with electrontherapy. A granulomatous reaction is, except in Hodgkin's disease, a rare phenomenon in lymphoproliferative disorders, particularly in cutaneous T cell lymphoma. This variant of mycosis fungoides raises the problem of the histological differentiation from other granulomatous dermatoses, mainly sarcoidosis. Its prognostic significance is disputed and its pathogenesis remains unknown.

Biopsy, Needle↗

PubMed1995

Treatment of advanced mycosis fungoides and Sézary syndrome with continuous infusions of methotrexate followed by fluorouracil and leucovorin rescue.

BACKGROUND AND DESIGN: The treatment of advanced mycosis fungoides is a therapeutic challenge. A variety of treatment approaches have been used. In our experience, chemotherapy has been most useful. The purpose of this study was to evaluate the effectiveness of the synergy previously demonstrated between methotrexate and fluorouracil in the treatment of advanced mycosis fungoides. Ten patients with mycosis fungoides and Sézary syndrome stages IIa (n = 1), II-b (n = 4), III (n = 1), IVa (n = 2), and IVb (n = 2) were treated with sequential methotrexate followed by fluorouracil and leucovorin rescue. Each patient received several courses of chemotherapy at varying intervals, as required for control of their disease. RESULTS: The duration of treatment ranged from 3 to 78 months, with an average duration of 33 months. The number of cycles of chemotherapy administered to each patient ranged from five to 45, with an average of 18 infusions per patient. The average survival in patients with tumors was 5.25 years, with a median survival of 6 years. Eight of 10 patients achieved at least 80% clearing and the remaining two achieved at least 60% clearing. Adverse reactions were minimal and included nausea and vomiting, mucositis, and leukopenia in only one patient. CONCLUSION: Sequential methotrexate and fluorouracil chemotherapy is an effective and safe treatment for advanced mycosis fungoides and Sézary syndrome. This regimen is extremely well tolerated, with minimal toxic side effects.

Adult↗

PubMedDate not supplied

Inflammatory vitiligo-like macules that simulate hypopigmented mycosis fungoides.

Two cases of an inflammatory vitiligo-like condition that simulated mycosis fungoides are reported. Both patients presented acquired hypopigmented macules sharply limited by an erythematous and papular border. The clinical aspect was suggestive of inflammatory vitiligo. Mycosis fungoides was suspected on skin specimens showing a dense band-like lymphocytic infiltrate with discrete nuclear atypias and marked exocytosis. This infiltrate was made of CD3 positive lymphocytes. CD8 positive lymphocytes were numerous in one case, few in the other. There was a loss of melanocytes in the lesional skin and absence of dominant T-cell clones in both cases. No repigmentation was observed after PUVA or local chemotherapy. The authors emphasized that erythematous and papular borders surrounding hypopigmented macules, CD8 positive lymphocytic infiltrate, absence of T-cell clonal rearrangement are helpful to rule out mycosis fungoides.

Adult↗