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PubMed1982

Mycosis fungoides d'emblée: a rare presentation of cutaneous T-cell lymphoma.

A case of the d'emblée variant of mycosis fungoides is presented to confirm the validity of this rare variant of cutaneous T-cell lymphoma. The patient had rapidly progressing cutaneous tumors of mycosis fungoides with no internal organ or nodal involvement at the onset of the disease. This was confirmed at a laparotomy, which was done to remove a uterine leiomyoma. The classic light microscopy was confirmed by electron microscopy. Postmortem findings showed a remarkable degree of "epitheliotropism." The d'emblée form of mycosis fungoides has been disputed by some who argue that these cases represent a lymphoma that begins elsewhere and has secondary skin involvement. In this patient there was an opportunity, within a few weeks of the onset of the problem, to do an extensive staging work-up, including laparotomy. No evidence of internal lymphoma was found.

Adult↗

PubMed1982

Spinal cord compression in mycosis fungoides.

Symptomatic involvement of the central nervous system (CNS) with mycosis fungoides is rare, and usually characterized by involvement of meninges. We describe a patient with long-standing mycosis fungoides who developed acute spinal cord compression. Since tumor-related spinal cord compression requires early intervention for a successful outcome, it should be recognized as an additional form of CNS mycosis fungoides.

Humans↗

PubMed1983

OKT 9 reactivity in mycosis fungoides and large plaque (atrophic) parapsoriasis.

A monoclonal antibody OKT 9 which detects a determinant expressed by a variety of proliferating cell types has been recently developed. This antibody was used in conjunction with the immunoperoxidase technique to study the cutaneous lymphoid infiltrates of nine patients with mycosis fungoides, one patient with lymphomatoid papulosis, two patients with Sézary syndrome, and ten patients with large plaque atrophic parapsoriasis (a condition which may terminate in overt mycosis fungoides.) OKT 9 reactive cells were identified in all cases of mycosis fungoides examined, in one case of lymphomatoid papulosis, one of two cases of Sézary syndrome, and one of ten cases of large plaque atrophic parapsoriasis. These results suggest that further studies using OKT 9 should be performed to assess whether OKT 9 reactivity may be used as a prognostic marker in cutaneous lymphomas and prelymphomas.

Antibodies, Monoclonal↗

PubMed1990

Epidermal Langerhans' cell densities influence survival in mycosis fungoides and Sézary syndrome.

Because Langerhans' cells (LC) (CD1a-positive epidermal cells) have been discussed to be involved in the pathogenesis of mycosis fungoides and Sézary syndrome, the authors examined the influence of densities of Langerhans' cells and, concurrently, of other phenotypes retrospectively on survival of 35 patients. Cell densities were assessed on cryostat sections (alkaline phosphatase antialkaline phosphatase-technique) of the respective diagnostic biopsy specimens. Additionally, two clinical parameters (age, stage of disease) were evaluated. CD1a-positive epidermal cells were demonstrated to be the only cell population being significantly associated (P = 0.011) with survival. Death resulting from mycosis fungoides and Sézary syndrome was significantly (P = 0.003) less frequent in patients with epidermal CD1a-positive cell densities higher than 90 cells/mm2 (optimal break point) as compared with patients with lower numbers. These results suggest that Langerhans' cells have a significant impact on prognosis of patients with mycosis fungoides and Sézary syndrome. They play an important role in the host defense mechanisms against these lymphomas rather than to favor their progression as proposed recently.

Adult↗

PubMed1983

Simultaneous occurrence of mycosis fungoides and Hodgkin disease: clinical and histologic correlations in three cases with ultrastructural studies in two.

We present three patients who manifested both Hodgkin disease and mycosis fungoides. Ages ranged from 39 to 66 and two were male. Skin lesions were present from 3 to 40 years before the diagnosis of Hodgkin disease. In all cases, mycosis fungoides was confirmed histologically by skin biopsy; the clinical course of the mycosis fungoides was indolent in all cases. Hodgkin disease was confirmed histologically in three, and confirmed by electron microscopy in two. All three patients responded to appropriate treatment for Hodgkin disease and are alive and well at the present time.

Adult↗

PubMed1987

Granulomatous reactions in mycosis fungoides.

Granulomatous reaction characterized by the formation of noncaseating accumulations of epithelioid histiocytes and multinucleated giant cells of the foreign-body type is a rare, poorly understood, and generally ignored phenomenon seen in various types of lymphoma. Its presence in cutaneous infiltrates of mycosis fungoides is equally unusual, but some favorable prognostic significance has been ascribed to it previously. In this paper, four patients with typical mycosis fungoides and granulomas demonstrated histologically in their cutaneous infiltrates are presented. All four died of disseminated disease with evidence of central nervous system involvement in three of them. These clinical histories lend no support to the notion that granulomatous mycosis fungoides is a benign variant of this lymphoma. Relevant literature is reviewed and discussed.

Aged↗

PubMed1977

[Age, duration of disease and survival period of patients with involvement of the nervous system by malignant lymphomas. A statistical comparison of mycosis fungoides with lymphogranulomatosis (author's transl)].

A statistical comparison dealing with age, duration of disease and survival period of patients with involvement of the nervous system by malignant lymphomas revealed the following data: 1. With mycosis fungoides, involvement of the nervous system occurs later in life than with lymphogranulomatosis; this difference is statistically highly significant (P less than 0.001). 2. There is no statistical difference (P greater than 0.05) in the duration of either type of malignant lymphoma before the nervous system becomes involved. 3. It is of high statistical significance (P less than 0.001) that, once the nervous system has become involved, the survival period is lower with mycosis fungoides than with lymphogranulomatosis. It can be expected that involvement of the nervous system by mycosis fungoides leads with 79% probability (confidential limits 99% = 47.29--96.22%) to death within 6 months.

Age Factors↗

PubMed1975

Immunoblastic sarcoma with leukemic blood picture in the terminal stage of mycosis fungoides.

A 76 year old man with mycosis fungoides developed an immunoblastic sarcoma and a leukemic blood picture in the final tumor stage after 6 years, in which the disease had clinically progressed in a typical manner. The results of histological and cytochemical studies of autopsy material are presented. Based on these findings and evidence of the T cell nature of mycosis fungoides, the immunoblastic sarcoma observed in the terminal stage of this case of mycosis fungoides might be of the rare T cell type.

Acid Phosphatase↗

PubMed1977

DNA content of mycosis fungoides cells.

DNA content of mycosis infiltrate cells was measured in 5 patients with the Feulgen cytophotometric method in the plaque and tumor stages. In addition, the infiltrate cells were differentiated cytochemically into histiocytes and atypical lymphoid cells with NaF-sensitive naphthol-AS-D-acetate esterase. In no case was an aneuploid stem line demonstrated. However, increasing duration of the tumor stage was associated with a larger proportion of tetraploid and octoploid cells. The DNA histograms also exhibited a local proliferation of atypical lymphoid cells. This proliferation was arrested by cytostatic therapy. Comparison with semi-thin-sections of tumor tissue showed that the mycosis fungoides cells are atypical lymphoid cells. These DNA measurements do not contradict the concept of limited aneuploidy, as reported in cytogenic studies. Thus mycosis fungoides fits in with the DNA distribution pattern in the group of lymphomas.

Adult↗

PubMed2001

[Follicular mycosis fungoides (FMF): a rare disease].

Follicular mycosis Fungoides (FMF) was first described in 1924. Since the first description, 22 patients with this special form of mycosis fungoides have been published. Clinical features include epidermal cysts as well as follicular papules, nodules and hyperkeratoses. FMF can be confused with acneiform dermatoses. In most cases, infiltrated plaques typical for cutaneous T cell lymphoma are also present. Histology shows a monomorphic CD4+ T cell infiltrate. Treatment and prognosis are similar of those of classical mycosis fungoides. We present one patient with FMF and review the literature of all published cases.

Acitretin↗

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↗

PubMed1991

A subpopulation of Langerhans cells (CD1a+Lag-) increased in the dermis of plaque lesions of mycosis fungoides.

The population of CD1a+ cells and the quantity of Birbeck granules were evaluated in comparison with the population of T lymphocytes in a variety of clinical lesions of mycosis fungoides. Anti-CD1a and Lag antibodies that specifically react with Birbeck granules and related structures of human Langerhans cells were used immunohistochemically. CD1a+ cells in the dermis of lesions of mycosis fungoides significantly increased in plaques of the plaque stage and in plaques of the tumor stage. They were most frequent in lesions with CD4+ cells ranging in number from 100 to 150/mm2. These lesions were suspected to be progressing from the plaque to the tumor stage. During the course of the disease, most of the dermal CD1a+ cells had few Lag antigens. These results suggest that dermal CD1a+Lag- cells may promote the progression of mycosis fungoides from the plaque to the tumor stage.

Antibodies, Monoclonal↗

PubMed1995

A clinical and histologic mycosis fungoides simulant occurring as a T-cell infiltrate coexisting with B-cell leukemia cutis.

One year after the onset of chronic lymphocytic leukemia, an elderly man had scaly cutaneous plaques on the thighs that clinically and histologically resembled the mycosis fungoides type of cutaneous T-cell lymphoma. Two years later the patient had indurated, red dermal nodules on the face that clinically and histologically were characteristic of cutaneous chronic lymphocytic leukemia. Immunophenotyping results from a facial nodule confirmed the presence of a B-cell infiltrate (CD20+). Immunophenotyping of a lesion on the right thigh showed that half the cells were composed of a CD2+, CD45RO+ (UCHL-1+) upper dermal and focally epidermotropic population of T cells consistent with mycosis fungoides; however, these T cells coexisted with an equal number of CD20+ B cells arranged in distinct clusters. DNA from the thigh lesion exhibited a B-cell immunoglobulin gene rearrangement, but the T-cell receptor gene rearrangements were germline. In this case, the evidence favors a mycosis fungoides simulant occurring as a reactive T-cell infiltrate to an underlying B-cell chronic lymphocytic leukemia.

Aged↗

PubMed1994

Prognosis with newly diagnosed mycosis fungoides after total skin electron radiation of 30 or 35 GY.

PURPOSE: To determine the prognosis of new patients with T1-4N0-1B0M0 mycosis fungoides treated with total skin electron beam radiation. METHODS AND MATERIALS: 25 consecutive patients received 30 Gy with 3 or 4 MeV electrons in 1977-1980; 121 received 35 Gy with 4 MeV in 1980-1992. Response rates, relapse-free survival, and overall and cause-specific survivals were assessed by explicit criteria. The relationships of T, N, gender, age, and radiation technique to prognosis were investigated by regression statistics. RESULTS: The average age was 55 years and the male:female ratio was 1:4. Forty-four percent were T1N0 and 34% were T2N0. The overall complete response rate was 82%, and lower T status, more radiation, and female gender were independently and positively associated with response. Median follow-up was 5.2 years. T1 patients who entered remission had a higher relapse-free survival compared to T2 through T4 patients. Thirty-four percent of T1 patients remained relapse-free at 6 years, compared to fewer than 20% of T2-4 patients. For all 146 patients the median overall survival was not reached at 15 years. Only 8 of 29 deaths were related to mycosis fungoides and these were significantly associated with higher T. The 54 T1N0 patients who had 35 Gy had a 10-year mycosis fungoides-specific survival of 100%. CONCLUSION: Total skin electron beam radiation gives good results with T1N0B0M0 disease. T3-4 disease is less likely to respond, it relapses more quickly, and it implies a poorer survival, but radiation offers palliation. T2 responds like T1, but relapses like T3-4. T2 also implies an intermediate survival. These results have implications for staging, informed consent, optimizing radiation treatment, and clinical trials.

Female↗

PubMed1991

A registry-based case-control study of mycosis fungoides.

The etiology of mycosis fungoides is unknown. Two possible causes (an unknown retrovirus with increased prevalence among never-married men, and prior malignancies) were investigated to determine whether they are associated with the incidence of mycosis fungoides. During 1973 to 1986, 953 case patients with mycosis fungoides or Sézary syndrome were registered by the Surveillance, Epidemiology, and End Results program. Each was matched by 5-year age group, sex, ethnicity, and geographic area to four control subjects, one each with cancer of the pancreas, brain, and stomach, and non-Hodgkin's lymphoma. For never-versus ever-married men, none of the relative risks differed significantly from those for women (odd ratios, .8-1.0). For any prior malignancy, the relative risks (and 95% confidence intervals) were 1.3 (.9-2.0), 1.2 (.8-1.8), 1.0 (.7-1.5), and 1.1 (.7-1.6). These data reject the previous relative risk estimate of 3.3 with greater than 99% power, and are consistent with only a small risk, if any, attributable to prior malignancy.

Aged↗

PubMed2004

An open trial of climatotherapy at the Dead Sea for patch-stage mycosis fungoides.

BACKGROUND: Climatotherapy at the Dead Sea (CDS) is a well-established therapeutic modality for moderate to severe psoriasis vulgaris, resulting in sustained remissions. It has also been found to be effective for atopic dermatitis, another T-cell-mediated dermatosis. OBJECTIVE: We sought to prospectively evaluate the efficacy of CDS in patch-stage mycosis fungoides. METHODS: A total of 12 patients with patch-stage mycosis fungoides (6 with stage IA and 6 with stage IB) were treated with CDS as monotherapy for 28 consecutive days according to the protocol for psoriasis, ie, a gradual increase of sun exposure to a maximum of 3 hours daily. RESULTS: A total of 9 patients achieved a complete clinical response (CCR), defined as no disease activity present; 2 achieved an almost CCR, defined as the reduction by more than 90% of disease activity; and 1 achieved a partial response, ie, reduction by more than 50% of disease activity. A CCR was achieved in all the patients with stage IA disease and in 3 of the 6 patients with stage IB disease. Of the 9 with a CCR, 6 also showed histopathologic clearing. Duration of the remissions, during which no therapy was allowed except for emollients, lasted from 2 to 9 months (mean: 5 months). No serious short-term side effects were recorded. CONCLUSION: CDS appears to be an effective, well-tolerated therapy for patch-stage mycosis fungoides.

Adolescent↗

PubMed2004

Cutaneous malignant melanoma in association with mycosis fungoides.

We retrospectively analyzed the first 461 cases entered into our cutaneous lymphoma database and found 285 cases of mycosis fungoides. We also identified 6 cases of malignant melanoma, all of which were found in patients with mycosis fungoides. The crude rate of melanoma in the general population in England, United Kingdom, in 1998 was 8.8/100,000 in men and 11.4/100,000 in women. The incidence of melanoma found in our cohort of patients with mycosis fungoides was far higher, and in 4 of the 6 patients cannot be explained on the basis of prior therapy. The reason for this association is unclear, but this report emphasizes the risk of second malignancies for patients with cutaneous T-cell lymphoma and melanoma.

Adult↗

PubMed2004

Clonal identity between skin and synovial tissue in a case of mycosis fungoides with polyarthritis.

Polyarthritis in the presence of a cutaneous T-cell lymphoma is a rare phenomenon. We describe a case of mycosis fungoides with development of a symmetric erosive polyarthritis of the small hand joints and feet, diagnosed as rheumatoid arthritis. An identical monoclonal T-cell population in the skin and in the synovium was detected by T-cell receptor gene rearrangement analysis, illustrating articular dissemination of lymphoma cells. Differentiating mycosis fungoides-associated arthritis from rheumatoid arthritis may have important implications for treatment. Based on this case, the relevant literature, and the newest disease concepts, pathogenic mechanisms and therapeutic options of mycosis fungoides-associated arthritis are discussed.

Arthritis, Rheumatoid↗