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PubMed1987

Successfully treated Hodgkin's disease followed by mycosis fungoides: case report and review of the literature.

We report the case of a 30-year-old man who had Hodgkin's disease of the nodular sclerosing type and subsequently developed mycosis fungoides. The Hodgkin's disease was treated with radiation therapy and chemotherapy, and the patient was in complete remission. Seven years later mycosis fungoides occurred and rapidly became progressive. Autopsy revealed that the mycosis fungoides involved multiple organs without any evidence of Hodgkin's disease. The possible significance of the association of these two diseases is presented.

Adult↗

PubMedDate not supplied

Morphologic and morphometric study of the two main cell lineages involved in mycosis fungoides: the lymphoid cells and the Langerhans cells.

A comparative light and electron microscopic study was carried out on skin biopsies from 5 mycosis fungoides patients (stages II and III). In addition, certain zinc-iodide-osmium (ZIO) procedures that selectively reveal the Langerhans cells (LC) were also applied. Several morphometric analyses, such as relative frequency, cell size, nuclear:cell ratios and nuclear contour indexes, were performed. Two main cell lineages were found in the dermal infiltrate: the lymphoid cells, represented by lymphoblastlike cells (LB), lymphocytes (L), and mycosis fungoides cells (MFC), and the Langerhans cells. A pattern of cellular organization of the dermal infiltrate was recognized. The LC appeared as the "organizing" cell. The morphometric study indicated that all LB, L, and MFC have the chance, at the same time, to be in contact with a LC. The application of the ZIO procedures clearly indicated a "translocation" of the LC, since they were scarce or absent from the epidermis and numerous in the dermis. Evidence is presented indicating that differentiation of LB into L and malignant transformation of L into MFC are processes occurring predominantly in the dermis. It is postulated that in the chronic stages of mycosis fungoides, L-LC contacts occur mainly in the dermis and not in the epidermis and that the development of malignant clones may occur in the dermis.

Aged↗

PubMed1982

Cranial computed tomography in the tumorous stage of mycosis fungoides.

Central nervous system (CNS) involvement by mycosis fungoides (MF) is rare. This is especially true of metastatic parenchymal involvement. Findings in two patients having the tumorous stage of MF are described. Multiple, well delineated, homogeneously and markedly enhancing nodules were seen in these two patients. The computed tomographic (CT) features of CNS involvement by MF are not specific. The differential diagnosis is discussed. By using CT in patients with MF, we will eventually be able to determine the incidence of CNS involvement in this condition.

Brain Neoplasms↗

PubMed1977

Topical mechlorethamine. Cutaneous changes in patients with mycosis fungoides after its administration.

Six patients with mycosis fungoides were treated with topical mechlorethamine hydrochloride for periods of two to four years. Clinical and histological studies for radiomimetic and radiodermatitis-like effects failed to demonstrate any abnormalities. The only observed changes were generalized hyperpigmentation of the skin and melanin-containing melanophages in the papillary dermis. We consider that the long-term use of topical mechlorethamine may be a safe form of therapy, but that a continuous indefinite follow-up of patients on this medication should be mandatory.

Administration, Topical↗

PubMed1998

Mycosis fungoides with involvement of the oral mucosa.

Mycosis fungoides (MF) is a T-cell lymphoma that predominantly affects the skin. Involvement of the oral tissues in MF is rare. A case of MF with involvement of the maxillary gingiva and cervical lymph nodes is presented. Electron-beam radiation with 40 Gy and systemic chemotherapy with vincristine and interferon gamma produced almost complete remission of the oral lesion while the persistent pain was relieved.

Antineoplastic Agents, Phytogenic↗

PubMed2000

Deafness from eighth cranial nerve involvement in a patient with large-cell transformation of mycosis fungoides.

Central nervous system (CNS) involvement by mycosis fungoides (MF) is rare and is usually seen in advanced stages with lymph node or visceral involvement. We describe a patient with advanced stage MF in large-cell transformation who presented with profound hearing loss after chemotherapy. Despite an initial differential diagnosis of vincristine-related neurotoxicity based on clinical, audiometric, and MRI investigations, CSF examination revealed lymphomatous leptomeningeal involvement. This case illustrates the importance of an awareness of the possibility of CNS involvement by MF and underlines the need for a complete neurologic evaluation including CSF examination in a patient with underlying MF who presents with a new neurological problem.

Aged↗

PubMed1992

[Observation of T cells and T-cell subsets in mycosis fungoides].

Skin lesions from 14 patients with mycosis fungoides (MF) were studied immunohistochemically with monoclonal antibodies that identify pan T (Leu4), T-helper/inducer (TH), T-suppressor/cytotoxic (Ts) (Leu3a, Leu2a) and pan B (Leu12) cells. Lymphoid cell reactivity was diverse among these cases. Among 12 cases of MF, TH cells were predominant over Ts cells, and one case featured nonreactive Ts cells in the dermis. In one case of MF, Ts cells were predominant. One case of MF showed strong reactivity for both T cell subset markers. The intraepidermal lymphoid cellularity was Leu4-reactive in 14 cases of MF. Among these 14 cases, the intraepidermal cellularity was Leu3a-reactive in 11 and Leu2a-reactive in 4. The use of such studies of T cell subsets may demonstrate a correlation of MF with cytomorphology, clinical stage, and disease prognosis.

Adult↗

PubMed2006

[Mycosis fungoides of the eyelids. Two case reports].

Mycosis fungoides is a distinct variant of cutaneous T cell lymphoma. We describe two male patients presenting with the third stage of the disease at the age of 56 and 67 years. The patients had large, patchy pigmented tumors all over the face and eyelids. Incisional biopsy from the lid skin revealed full-thickness involvement of the dermis by malignant lymphocytes, angiotropism, and rare figures of epidermotropism. Immunohistochemical studies showed that these malignant lymphocytes expressed CD2, CD3, CD4, CD5, and CD8 antigens but were negative for CD7 antigen. Multiagent chemotherapy and PUVA treatment were administered with limited successful outcome on their cutaneous lesions. Both patients died from problems related to the progression of the systemic disease. These two cases demonstrate the poor prognosis of the tumor stage because of strong association with widespread systemic involvement and emphasize the need for early diagnosis.

Aged↗

PubMed1993

Total skin electron irradiation: efficacy in early mycosis fungoides.

The rare, indolent, but lethal malignancy, mycosis fungoides (MF), is amenable to durable remissions if treated topically at an early stage with nitrogen mustard, PUVA, or radiotherapy. A modification of conventional therapeutic irradiation which utilizes electron beams rather than photons, has been in use since 1951. This method, termed total skin electron irradiation (TSEI), has achieved consistently good CR rates (95-100%) at a variety of centres in the U.S.A., England, France, and Italy, despite troublesome differences in staging systems. In northern Israel we have treated 37 MF patients with TSEI during the past 13 years. All 21 of our early stage patients achieved CR, which is no longer regarded as an unusual result. However, most workers in the field acknowledge that issues of optimal dosing and curative potency remain unresolved.

Adult↗

PubMed2001

[Interferon-alpha and PUVA therapy for mycosis fungoides].

14 patients suffering from early stage mycosis fungoides were treated with interferon alpha 2-a and PUVA/1 patient in stage I a, 3 patients in stage I b, 4 patients in stage II a and 6 patients in stage II b/during 3-21 months time course. Interferon alpha 2-a was administered 3 times a week, in escalating dose from 3 MU to 9 MU, determining the individual maximal tolerated dose. All of the patients responded well to the treatment. Partial remission was observed after 4-13 weeks of treatment. Total remission developed in 8 cases, after 8 weeks- 9 months of the treatment. Side effects occurred frequently: weight loss, pain, fever, fatigue, leucopenia, thrombopenia, liver enzyme elevation. Because of the side effects the dose of the interferon was reduced individually, the dose reduction did not cause relapse.

Aged↗

PubMed1999

Follicular mycosis fungoides.

We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa.

Aged↗

PubMed1999

How useful are T-cell receptor gene rearrangement studies as an adjunct to the histopathologic diagnosis of mycosis fungoides?

The diagnosis and differential diagnosis of mycosis fungoides (MF) is often difficult, clinically and histologically. Recent attempts to enhance diagnostic sensitivity have involved T-cell receptor (TCR) gene rearrangement studies, using Southern blot or polymerase chain reaction (PCR) technique. PCR is more widely used because of its increased sensitivity, lower labor- and cost-intensive analytic steps, lack of radioactive substances, and applicability to routinely processed biopsies. Several studies that use this technique detect clonal bands in 50% to 83% of the MF specimens from patch, patch/plaque, and erythrodermic stages, which often pose a diagnostic challenge. This compares with no clonality in the control groups of most of these studies, or with up to 13% in a few studies, although long-term follow-up reveal that some cases of so-called "clonal dermatitis" eventually progress into overt cutaneous T-cell lymphoma. Furthermore, retrospective studies on archival histologic material from patients with MF demonstrate similar rates of clonality in histologically "inconclusive," "borderline," and diagnostic MF biopsies. Therefore, in the proper clinicopathologic setting, and with consideration of the known limitations of this technique, TCR gene rearrangement studies on lesional skin using PCR may be helpful as an adjunct to the histopathologic diagnosis of MF.

Diagnosis, Differential↗

PubMed1979

Clinical and immunological evaluation of 5 cases of mycosis fungoides in advanced stages.

Five patients with mycosis fungoides, hospitalized in the Division of Radiotherapy and Medical Oncology of the Ospedale Civile, Pordenone, from January 1975 to December 1978, were studied and treated as non-Hodgkin lymphomas. All patients had evidence of disseminated disease: 3 with bone marrow infiltration, 1 with splenic involvement and 1 with lymph node involvement. Three patients were treated with CVP, resulting in 2 complete remissions that lasted 18 months and 1 PR greater than 50% maintained for 7 months. One patient was treated with ABVD with a PR greater than 50% maintained for 10 months. The last patient was treated with prednisone and then with CV, but expired from pulmonary embolism after 1 cycle. Lymphocyte function, using E and EAC rosette and PHA, was evaluated before therapy in all patients: in the 2 patients who obtained a CR, an improvement in T-lymphocyte function was noted after therapy. The chromosome pattern of peripheral blood lymphocytes was altered before therapy in only one patient. Even if the follow-up period is still relatively brief, the duration of the 2 complete remissions must be stressed. In addition, a strict correlation between T-lymphocyte function and response to therapy was revealed in our study.

Adult↗

PubMed1999

Evidence for the cofactor role of human T-cell lymphotropic virus type 1 in mycosis fungoides and Sézary syndrome.

The aetiology of mycosis fungoides (MF) and Sézary syndrome (SS) is unknown. A pathogenic role for the human T-cell lymphotropic virus type 1 (HTLV-1) has been suggested but remains controversial. We used an animal model to test the possibility that peripheral blood mononuclear cells (PBMC) obtained from MF patients harbour the HTLV-1 virus which may be infective. The polymerase chain reaction (PCR) was used to detect HTLV-1 proviral DNA sequences in PBMC of 27 MF patients and one SS patient of non-Iranian origin. Positive results were found in six of the patients. Twelve of the 28 patients tested by Western blot showed HTLV-1 antibodies. Twenty-eight immunosuppressed inbred Fisher F344 rats were inoculated intravenously with cultures of PBMC obtained from the 28 patients. Eight of these 28 rats showed antibodies to HTLV-1 while the proviral genome was demonstrated in the blood of only two of the rats. PBMC from two MF patients, in spite of showing negative results for the proviral genome by PCR, still induced HTLV-1 antibody formation in the F344 rat model. None of 10 control rats inoculated with normal donor PBMC showed antibodies to HTLV-1, nor the proviral genome. The present study suggests that HTLV-1 plays a cofactor role in MF/SS patients.

Adoptive Transfer↗

PubMed2003

Systemic chemotherapy for the treatment of mycosis fungoides and Sézary syndrome.

The treatment of mycosis fungoides and Sézary syndrome is unique. The treatments of choice are highly stage-and practitioner-dependent. For early stage patients, treatment with palliative topical therapies is often adequate to yield excellent, high-quality and durable responses. For the more advanced stages, systemic approaches are more appropriate, either alone or in combination, to palliate patients. There is still little convincing evidence from randomized trials that any single approach is favorable for improving survival. Many practitioners believe the disease can be controlled with the host immune system, but there again is little scientific support for this conclusion. It has been hypothesized that some of our therapies work through this mechanism, such as photopheresis and perhaps even psoralen and ultraviolet A. Unfortunately, even the use of biologic response modifiers, such as interferon and the interleukins, is not absolutely supportive of an active role for immune surveillance since these agents have been shown to have cytotoxic and differentiating effects. Because of the above, many practitioners have actively discouraged the use of chemotherapy because it may impair the host immune system; certainly, the purine analogs would fall into this category. However, there is little evidence supporting significant immune effects for other chemotherapeutics, and clearly, the responses seen would support their use in appropriate patients. As the present authors will detail in this paper, the advances in understanding cancer biology and mechanisms of resistance should, in the future, lead to optimal selection of agents that are predicted to be optimally active, limiting the toxicity and waste associated with ineffective drug usage.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2006

Microscopic study of normal skin in cases of mycosis fungoides.

BACKGROUND: During therapy of patients with mycosis fungoides (MF) at the Department of Dermatology, Kasr El-Aini Hospital, follow-up biopsies are routinely taken every 2 months. It was noticed that lesions of MF might become clinically normal during treatment, and yet still show microscopical evidence of MF. This finding raised the possibility that clinically normal skin in MF could be microscopically involved. AIM: The aim of our work was to evaluate the degree of histopathological involvement of normal-looking skin in patients with MF. PATIENTS AND METHODS: Thirty patients with stage IB were biopsied from their normal skin. Two biopsies were taken: one proximal (2 cm) and the other distal (> 5 cm) from any visible lesion. Ten normal controls were included in the study. All specimens were stained with H&E and examined microscopically. The microscopical diagnosis was confirmed by immunophenotyping. RESULTS: Epidermotropism was detected in 21 (70%) of the proximal skin biopsies and 14 (47%) of the distal skin biopsies, whereas no biopsy from the control group showed epidermotropism. All the proximal skin biopsies showed dermal infiltrate and 90% of the biopsies from the distal normal skin showed dermal infiltrate (mostly superficial perivascular). CONCLUSION: Normal skin in patients with MF could be affected microscopically and this may raise questions regarding the credibility of the current staging classification of MF, and may necessitate taking biopsies from normal skin before starting topical treatment. During MF treatment, biopsies from cured lesions are required before starting withdrawal.

Adult↗

PubMed2003

Assessment of histologic criteria in the diagnosis of mycosis fungoides.

BACKGROUND: The histologic diagnosis of early mycosis fungoides (MF) can be difficult to establish in many instances because the subtle changes observed in patches of MF are also present in many inflammatory dermatoses. METHODS: To assess the frequency and significance of many of these histologic parameters, we retrospectively reviewed 50 slides from patients with documented MF in patch, plaque, and tumor stages. The diagnosis of MF was unequivocally established either by the progression of patients to advanced stages of the disease or by indubitable histologic findings. In the second phase of the study, we compared the histologic parameters observed in 24 patch stage MF patients with those in 24 non-MF patients. The non-MF group were patients whose pathologic pattern was suspicious for MF, but who definitely did not have MF on clinical grounds. The two groups were matched by histologic pattern. Two different observers evaluated the slides and the intensities of 32 histologic parameters were graded on a four-point scale to minimize the subjective variability in the histologic reports. RESULTS: On univariate analysis, the following parameters achieved significance in distinguishing MF from non-MF: Pautrier's microabscesses, haloed lymphocytes, disproportionate epidermotropism, epidermal lymphocytes larger than dermal lymphocytes, hyperconvoluted lymphocytes in the epidermis and dermis, absence of dyskeratosis, and papillary dermal fibrosis. None of these features proved to have additional discriminating power on multivariate analysis. CONCLUSIONS: The efficacy of single histologic features in the diagnosis of early MF is generally poor and, to discriminate MF from its inflammatory simulators, a combination of cytologic and architectural features must be used.

Adolescent↗

PubMed2002

Clonal heterogeneity in mycosis fungoides and its relationship to clinical course.

Mycosis fungoides (MF) is a cutaneous T-cell lymphoma characterized by multifocal disease and protracted clinical course. The few studies that have assessed T-cell receptor (TCR) gene rearrangements (GRs) present at different anatomic sites in MF have generally reported a common clone. We used a previously validated 4-color polymerase chain reaction (PCR) assay to assess the size and V-family usage of TCR-gamma GRs in 102 concurrent and/or sequential morphologically involved biopsy specimens (91 skin and 11 lymph nodes) from 39 MF patients. This assay detected TCR-gamma clonal GRs in 89 samples (87%) from 36 patients (92%). In 24 patients (77%), an identical clonal GR was present in at least 2 skin samples. However, in one third of these patients, additional different clonal GRs were also noted. Four patients (13%) had clonal GRs that were distinct in different skin samples. In 3 patients (10%), no GR was detected in any sample. In a comparison of lymph node and skin samples, 8 patients had the identical clonal GRs at both sites, 2 patients had different clonal GRs, and 1 patient had no GR identified at either site. Independent of clinical stage, patients who had the same GR detected in multiple concurrent biopsy specimens at the time of diagnosis were more likely to have progressive disease than those who had different GRs (P =.04). Four-color TCR-gamma PCR analysis can uncover multiple distinct clonal GRs in different samples consistent with multiclonal or oligoclonal disease in a significant proportion of MF patients. Demonstration of identical clonal GRs in multiple biopsy specimens at the time of diagnosis may provide prognostic information related to disease progression.

Adolescent↗