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PubMed2005

Defining early mycosis fungoides.

This editorial review summarizes the results of 5 meetings sponsored by the International Society for Cutaneous Lymphoma at which the clinicopathologic and ancillary features of early mycosis fungoides were critically examined. Based on this analysis, an algorithm was developed for the diagnosis of early mycosis fungoides involving a holistic integration of clinical, histopathologic, immunopathologic, and molecular biological characteristics. A novel aspect of this algorithm is that it relies on multiple types of criteria rather than just one, for example, histopathology. Before its finalization, the proposed diagnostic algorithm will require validation and possibly further refinement at multiple centers during the next several years. It is anticipated that a more standardized approach to the diagnosis of early mycosis fungoides will have a beneficial impact on the epidemiology, prognostication, treatment, and analysis of clinical trials pertaining to this most common type of cutaneous lymphoma.

Algorithms↗
PubMed2005

[Follicular mycosis fungoides, comedo-like and cystic].

Follicular mycosis fungoides is an infrequent variant of mycosis fungoides. It has classically been defined by the presence of an atypical lymphoid infiltrate around and in the follicular epithelium with little or no epidermotropism, and no follicular mucin deposits. The fact that there are cases with epidermal involvement and/or follicular mucinosis means that some uniform diagnostic criteria are necessary. We describe two cases of follicular mycosis fungoides with follicular mucinosis and with varying degrees of associated epidermotropism.

Adult↗
PubMed1984

Morphologic heterogeneity of malignant lymphomas developing in mycosis fungoides.

From an extensive series of patients with mycosis fungoides, we identified 12 in whom subsequently developing extracutaneous (lymph nodal) lymphoma manifested morphologic features other than those of so-called cutaneous T-cell lymphoma. Six patients had features diagnostic of Hodgkin's disease, two had morphologic and cytochemical features consistent with T-cell lymphoma but without the morphologic features ascribed to cutaneous T-cell type, and four had morphologic characteristics most consistent with B-cell lymphoma. Although in most cases of mycosis fungoides the lymphomas exhibit morphologically distinctive features of mycosis cells, we propose that in occasional cases this morphologic correlation is not present.

Adult↗
PubMed1984

Treatment of parapsoriasis and mycosis fungoides: the role of psoralen and long-wave ultraviolet light A (PUVA).

Psoralen and long-wave ultraviolet light A (PUVA) was used to treat 12 patients with parapsoriasis and 19 patients with mycosis fungoides. Excellent results were obtained in all 12 patients with parapsoriasis: 8 patients had complete clearing, 3 had more than 80% improvement, and 1 had considerable improvement. The response to PUVA in patients with mycosis fungoides varied according to the stage of the disease. Of 10 patients with early disease (stage I or II), 7 had complete clearing and the 3 others had more than 80% improvement. The nine patients with more advanced disease generally did not respond well to PUVA, with the exception of one patient with stage IV disease and one with stage V disease. PUVA may be the treatment of choice for parapsoriasis and is highly effective in the early stages of mycosis fungoides.

Female↗
PubMed1985

Common activated helper-T-cell origin for lymphomatoid papulosis, mycosis fungoides, and some types of Hodgkin's disease.

A hypothesis is proposed to explain the nature of the Reed-Sternberg (RS) cell and the unexpectedly common clinical associations between lymphomatoid papulosis, mycosis fungoides, and some types of Hodgkin's disease. The RS cell appears to be a lymphoblast arising from activated helper T cells. In lymphomatoid papulosis, a cutaneous eruption with histological features of Hodgkin's disease, there is a spectrum of activated helper T cells, including cerebriform cells and large transformed RS-like cells. Clonal expansion of cerebriform cells in lymphomatoid papulosis leads to mycosis fungoides. Similar expansion of large transformed cells results in Hodgkin's disease. Progressive transformation of T4-positive cerebriform cells to Ki-1-positive RS cells accounts for rare cases of coexistent mycosis fungoides and Hodgkin's disease. Confirmation of this hypothesis should focus attention on the family of human T-cell leukaemia/lymphoma viruses as possible aetiological agents in these helper-T-cell disorders.

Antigens, Neoplasm↗
PubMed1999

Radiotherapy in the management of mycosis fungoides: indications, results, prognosis. Twenty years experience.

PURPOSE: To determine the role of total skin electron beam therapy (TSEBT) and the prognosis of patients with mycosis fungoides. MATERIALS AND METHODS: From 1978 to 1996, 66 consecutive patients with mycosis fungoides received 30 Gy TSEBT delivered in 12 fractions over 40 days as treatment of their relapsed after topical or/and systemic therapy. All patients were staged as follows: stage A, superficial lesions covering less than 50% of the body surface; stage B, superficial lesions covering more than 50% of the body surface; Stage C. tumors involving the skin, lymph nodes and/or visceral organs. RESULTS: The median age was 50 years (ranging from 13-78 years). There were 39 males and 27 females. The minimum follow-up was 12 months (range 12-192 months). There were 24 (36%) stage A patients, 22 (33%) stage B patients, and 20 (30%) stage C patients. The overall survival at 5 years for our series was as follows: 93% for stage A; 79% for stage B, and 44% for stage C disease (P = 0.002). For the entire cohort, the complete remission rate was 65%, the progression-free survival (PFS) at 5 years and 10 years was 30 and 18% respectively. For the group A, PFS was 62% at 5 years and 46% at 10 years; for group B, PFS was 19% at 5 years. CONCLUSIONS: This study suggests that TSEBT gives good results for relapsed stage A disease. Total skin electron beam therapy combined with local fields irradiation or other local treatment (puvatherapy or topical nitrogen mustard) might further improve permanent complete response for stage B patients. The most advanced stages of mycosis fungoides are not controlled by TSEBT, but the radiation therapy offers good palliation results.

Adolescent↗
PubMed2000

Hypopigmented mycosis fungoides: treatment and a 6(1/2)-year follow-up of 9 patients.

BACKGROUND: The clinical course of hypopigmented mycosis fungoides has not been well described. OBJECTIVE AND METHODS: We sought to review the management and follow-up of patients with hypopigmented mycosis fungoides evaluated between January 1990 and August 1998. RESULTS: There were 7 African American and 2 Asian patients (5 male and 4 female patients); 8 had stage Ia and 1 had stage Ib disease. The mean age at diagnosis was 34.4 years, the mean latent period was 9.2 years, and the mean follow-up period after diagnosis was 6.5 years. Treatment modalities used included psoralen UVA, UVB, and topical mechlorethamine. Eight had complete clinical responses, and another had a partial response. The remission period ranged from 2 months to 3 years. In all but one patient, lesions recurred; all responded rapidly to another course of therapy. CONCLUSION: Hypopigmented mycosis fungoides is characterized by early onset, occurrence in dark-skinned individuals, and good response to therapy. Recurrences are common. In most, it has a biologically benign course.

Adolescent↗
PubMed2003

Low-dose methotrexate to treat mycosis fungoides: a retrospective study in 69 patients.

BACKGROUND: Although low-dose methotrexate has been used to treat mycosis fungoides for many years, documentation is very limited. OBJECTIVE: Our purpose was to review our experience with methotrexate in the treatment of 69 patients with patch/plaque and tumor stage mycosis fungoides observed for up to 201 months. METHODS: This was a retrospective study. Data are presented in terms of response rates and time to treatment failure. RESULTS: The greatest number of patients (60) had patch/plaque stage T2 disease (>/=10% skin involved). Of these, 7 (12%) achieved complete remission and 13 (22%) achieved partial remission for a total response rate of 20 of 60 (33%). The median time to treatment failure was 15 months. Only 1 of 7 patients with tumor stage disease responded. Side effects caused treatment failure in 6 (9%) of the total cohort of 69 patients. CONCLUSION: Low-dose methotrexate may be of value in the treatment of a subset of patients with patch/plaque mycosis fungoides resistant to other therapies.

Adult↗
PubMed1979

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↗
PubMed1982

Controversies in mycosis fungoides.

Most clinicians agree that mycosis fungoides is the prototypic cutaneous T cell lymphoma. However, certain clinical characteristics indicate that this disorder may begin as a reactive rather than a neoplastic process. The concept of a nonneoplastic etiopathogenesis of mycosis fungoides is further supported by recent data on the function of Langerhans cells, a population of epidermal cells known to play a critical role in immune surveillance and the development of contact sensitivity. It has been suggested that chronic occupational exposure to environmental allergens results in persistent antigenic stimulation, leading to a breakdown in immune surveillance and eventually, malignancy. Modern laboratory technics have enhanced the clinician's ability to diagnose and stage mycosis fungoides. Data obtained from such studies have indicated that systemic spread may occur much earlier in the course of disease than has previously been appreciated. The therapeutic implications of such knowledge are as yet uncertain.

Allergens↗
PubMed1984

Monoclonal gammopathy and mycosis fungoides. Report of four cases and review of the literature.

Of four patients with mycosis fungoides and monoclonal gammopathy, one died of multiple myeloma that developed 4 years after the mycosis fungoides. The other three had monoclonal gammopathy of undetermined significance. This association does not appear to occur with a high frequency in patients with mycosis fungoides than in the general population. However, it demonstrates that B cell proliferation can occur in patients with primary cutaneous T cell disease.

Aged↗
PubMed1985

Remission in skin infiltrate of a patient with mycosis fungoides treated with cyclosporine.

A 57-year-old woman with mycosis fungoides that had failed to respond to cytotoxic chemotherapy was treated with cyclosporine. Mycosis fungoides and Sézary syndrome are disorders of helper T cells. Cyclosporine is a fungal endecapeptide of novel chemical structure that causes preferential inhibition of T helper cells. Because of this in vitro inhibition of T helper cells, we used cyclosporine to treat a patient who had mycosis fungoides that was refractory to cytotoxic combination chemotherapy. With cyclosporine administered initially as an intravenous infusion and orally after 10 days, there was immediate improvement in the patient's symptoms. This subjective improvement was accompanied by a decrease in her skin infiltration, noted on physical examination and microscopically. Despite continued administration of the cyclosporine, symptoms recurred after 3 1/2 months of therapy.

Cyclosporins↗
PubMed1987

Mycosis fungoides beginning in childhood and adolescence.

Mycosis fungoides is a form of cutaneous T cell lymphoma usually occurring in mid to late adulthood. We report 12 patients with mycosis fungoides whose eruption began before age 20 years, including one patient with histologic documentation at 5 years of age. The onset of mycosis fungoides during the first two decades may be more common than is generally recognized and should be included in the differential diagnosis of chronic dermatoses in children and adolescents.

Adolescent↗
PubMed1988

Progression of pigmented purpura-like eruptions to mycosis fungoides: report of three cases.

The cases of three relatively young male patients with pigmented purpura-like eruptions evolving to mycosis fungoides are described. Initially, the eruptions, both clinically and histologically, very closely resembled pigmented purpuric dermatitis. After a follow-up period averaging 8.4 years, diagnostic histologic changes of mycosis fungoides were observed. Although uncommon, mycosis fungoides should be considered in the differential diagnosis of pigmented purpuric eruptions.

Adolescent↗
PubMed1994

Emergence of leprosy in a patient with mycosis fungoides.

A patient with mycosis fungoides that had progressed to tumor stage responded to chemotherapy and electron beam treatment, but 6 years later a peripheral neuropathy, extensive plaques, erythroderma, and enlarged pinnae containing acid-fast organisms developed while he was being treated with photopheresis. The skin lesions cleared with administration of rifampin and dapsone, but a reversal reaction biopsy specimen showed features of both mycosis fungoides and leprosy. This case raises the question of whether there may be an association between mycosis fungoides and leprosy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

Antitumor activity of DAB389IL-2 fusion toxin in mycosis fungoides.

BACKGROUND: DAB389IL-2 is a novel fusion toxin that retargets the cytotoxic A-chain of diphtheria toxin to interleukin-2 (IL-2) receptor-expressing tumors. OBJECTIVE: The purpose of this phase I trial was to study the toxicity, maximum tolerated dose, and clinical efficacy of DAB389IL-2 in IL-2 receptor expressing lymphoproliferative malignancies, including cutaneous T-cell lymphoma. METHODS: DAB389IL-2 was administered intravenously daily for 5 days every 3 weeks. Dose escalation occurred between patient groups. Patients were monitored for laboratory and clinical toxicity, kinetics, immune response, and clinical efficacy. RESULTS: Thirty-five patients with cutaneous T-cell lymphoma (including 30 patients with mycosis fungoides) were treated. Previously, conventional therapy had not worked for 34 of the patients. Thirteen patients (37%) achieved an objective response, including a complete response in five patients (14%). Complete response was achieved in patients with extensive erythroderma and tumor stage mycosis fungoides. Adverse events consisted of reversible fever/chills, hypotension, nausea/vomiting, and elevation of hepatic transaminase. Doses of less than 31 microg/kg per day were well tolerated. Clinical responses were observed at all dose levels. CONCLUSION: DAB389IL-2 is well tolerated at doses of less than 31 microg/kg per day, and it induced clinical responses in previously treated mycosis fungoides, providing evidence for the antitumor activity of this molecule.

Adult↗
PubMed2001

[Transformation of mycosis fungoides to pleomorphic T-cell lymphoma and central nervous system involvement].

INTRODUCTION: Although mycosis fungoides is a malignant T-cell lymphoma involving mainly the skin, neurological complications are possible, with a poor prognosis. EXEGESIS: A 59-year-old man, treated for mycosis fungoides with transformation to a pleomorphic T-cell lymphoma for 1 year, was seen for mental status changes with confusion. A brain parenchyma localisation was found. CONCLUSION: This observation emphasizes the exceptional neurological tropism in the patients with mycosis fungoides. A transformation to a more aggressive cutaneous T-cell lymphoma seems necessary to induce a central nervous system involvement.

Brain Neoplasms↗
PubMed1998

Local superficial radiotherapy in the management of minimal stage IA cutaneous T-cell lymphoma (Mycosis Fungoides).

PURPOSE: To evaluate the impact of local superficial radiotherapy with respect to local control, survival, and toxicity for patients with "minimal" stage IA cutaneous T-cell lymphoma (Mycosis Fungoides). METHODS AND MATERIALS: Between 1954 and 1996 a total of 21 patients were identified as receiving curative local superficial radiation (LSR) for minimal stage IA Mycosis Fungoides. All patients had pathologic documentation at diagnosis and at the time of suspected recurrences and no patient received prior radiation. Ten patients were treated with 100-280 Kv (A1), and 11 with 4-12 Mev electrons. Nine patients had failed prior therapies (steroids: 4; PUVA: 3; BCNU: 1; UVB: 1) and six received adjuvant therapy after completion of LSR (PUVA: 5; steroids: 1). Minimum follow-up was 1 year. RESULTS: The median follow-up was 36 months (13-246), and the median age when commencing LSR was 55 years (27-73). All patients were Caucasian, and 11 were male. A total of 32 lesions were identified in 21 patients; 13 patients had unilesional disease, 5 patients had 2 lesions, and 3 had 3 lesions. A total of 33 fields were treated with a median treatment surface area of 107 cm2 (11-785). The median surface dose was 20 Gy (6-40), with 17 patients receiving a dose > or = 20 Gy. The median fraction number was 5 for all fields, but was 10 for the fields receiving 20-40 Gy. The complete response rate was 97%, and all patients were alive at last evaluation. All failures were cutaneous. One patient had persistent disease (treated with 6 Gy), and three failed locally at 52 months (8 Gy), 16 months (20 Gy), and 4 months (20 Gy). None of these patients received adjuvant therapy. Two patients failed in distant skin sites and were salvaged. The actuarial DFS for the entire group at 5 and 10 years was 75 and 64%, respectively, with local control of 75% at both time intervals. For the 13 patients with unilesional disease, the DFS was 85% at 10 years. For those treated with doses > or = 20 Gy, the DFS was 91% as was local control (no distant failures). Toxicity included mild erythema and dry desquamation acutely. Chronic toxicity included dermatitis [2], and telangiectasia [1]. No second cutaneous malignancies or hematologic toxicity was noted. CONCLUSION: Patients with minimal Stage IA Mycosis Fungoides may be managed effectively with local superficial radiation alone without adjuvant therapy. Distant failure is unusual and patients should receive a minimum surface dose of 20 Gy, which offers excellent local control. Sequalae of therapy are minimal.

Adult↗