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Immunogold detection of CD3 and CD4 antigens in patients with Mycosis fungoides.

In this study, we analyzed the distribution of CD3 and CD4 antigens at the ultrastructural level in tissue samples from mycosis fungoides patients using double-immunogold labeling. We observed clusters composed of CD3 and CD4 antigens on the plasma membrane and intracellular. There were also clusters only of one type of the antigen and we could observe more often CD4 than CD3. Labeling of CD3 and CD4 was not found in control cells incubated with non-immune serum. In conclusion, our ultrastructural studies not only visualized pattern distribution and relationship between CD3 and CD4 antigens but might also suggest that the type and form of distribution provides new clues to their possible translocation in mycosis fungoides cells.

CD3 Complex↗

Folliculotropic mycosis fungoides with central nervous system involvement: demonstration of tumor clonality in intrafollicular T cells using laser capture microdissection.

BACKGROUND: Folliculotropic mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma, MF type, characterized by atypical lymphocytes preferentially infiltrating the hair-follicle epithelium relative to the epidermis. OBSERVATIONS: We describe a rare case of folliculotropic MF involving the central nervous system. This is also the first case in which laser capture microdissection was used to show that the atypical lymphocytes within the hair-follicle epithelium were part of the same tumor clone present in other tissue compartments. CONCLUSIONS: In reviewing the literature describing atypical lymphocytes infiltrating hair-follicle epithelium relative to the epidermis, we encourage the use of the term folliculotropic mycosis fungoides. Our case also supports previous findings that central nervous system involvement can occur in advanced MF. The successful procurement and analysis of atypical lymphocytes from hair-follicle epithelium by laser capture microscopy ushers in a new era in molecular diagnostics.

Aged↗

Pseudolymphoma with mycosis fungoides manifestations, hyperresponsiveness to diphenylhydantoin, and lymphocyte disregulation.

Three patients receiving diphenylhydantoin (DPH) were seen with a reversible process suggesting mycosis fungoides. Clinical and laboratory manifestations included generalized pruritic exfoliative erythroderma, eosinophilia, lymphadenopathy, hepatosplenomegaly, circulating Sézary cells, epidermal Pautrier's microabscesses on skin biopsy, and moderate liver dysfunction. Studies of the distribution and function of the various lymphocyte subpopulations from these patients showed: (1) an increase in the relative and absolute number of T lymphocytes (85--92%); (2) significant stimulation of lymphocyte-blastic transformation by DPH and low response to pokeweed mitogen stimulation; (3) the impaired ability of T gamma lymphocytes to suppress B-cell differentiation and immunoglobulin production. With only one exception, 15 symptom-free patients on DPH showed none of these abnormalities. The clinical manifestations and immunologic abnormalities of patients with this pseudo mycosis fungoides syndrome remitted three to four weeks after DPH administration was discontinued. The proliferation of T lymphocytes and the inhibition of the function of T gamma-suppressor lymphocytes noted in these patients may be significant to the development of other types of pseudolymphoma and to that of true lymphoma.

Adolescent↗

Vesicular mycosis fungoides.

Blistering is not a feature normally associated with mycosis fungoides (MF). We present a case of MF in which histopathological vesicle formation was such a prominent feature that diagnosis was delayed. The patient's disease ran an aggressive course and death occurred within one year of presentation. Tumour involvement of the tongue with MF was an unusual late feature.

Blister↗

Light sensitivity in mycosis fungoides.

Because of suspected photosensitivity, twelve patients suffering from mycosis fungoides have been phototested with various wavebands. Four patients experienced a burning sensation and erythema following sun-exposure, after the disease had progressed to stage II. On phototesting, abnormal reactions were observed in 7 patients. The offending wavebands were situated mostly in the UVA range, but also in the UVB range and in visible light.

Adult↗

Combined chemotherapy (COP) in treatment of mycosis fungoides: report of four cases.

Four consecutive patients with mycosis fungoides received cyclic chemotherapy using cyclophosphamide, vincristine, and prednisone (COP). In one case, there was complete remission of disease for 15 months. In another, there was 50% regression of tumors with healing of the ulcerated surfaces. The third patient showed complete clearing of scaling and redness of the skin. In the fourth patient, the lesions progressed.

Aged↗

Purely follicular mycosis fungoides without mucinosis: report of two cases with review of the literature.

Follicular lesions can either associate with typical patch-/plaque-type mycosis fungoides or, more rarely, be the only clinical manifestation of the disease. We describe 2 adult patients who presented with alopecia and disseminated follicular erythematous papules, and comedones and cysts, respectively. In both patients, histology showed a folliculotropic infiltrate of atypical lymphocytes that spared the epidermis, in the absence of follicular mucinosis. Molecular genetic analysis confirmed the oligo/monoclonal nature of the T-cell infiltrate. Reported cases of purely follicular mycosis fungoides without mucinosis are reviewed.

Aged↗

Sarcoidosis associated with mycosis fungoides.

In November 1966, a 47-year-old black woman had a skin eruption and an abnormal chest roentgenogram that were found to be due to sarcoidosis. In September 1974, nodular lesions and subsequently generalized exfoliative dermatitis developed that were found to be due to mycosis fungoides. The concurrence of sarcoidosis and mycosis fungoides has been recorded only once in the literature. The depression of cell-mediated immunity found in this patient and other sarcoid patients suggests that impairment of the host's immunosurveillance system may predispose to the development of T-cell lymphomas.

Female↗

Minimal change glomerulonephropathy and interstitial infiltration with mycosis fungoides.

The nephrotic syndrome developed in a patient with mycosis fungoides shortly after systemic involvement by his tumor occurred. Renal biopsy examination revealed atypical lymphocytic interstitial infiltration and changes consistent with minimal change glomerulonephropathy. The patient's proteinuria decreased following steroid therapy. This is the first report of an association between minimal change glomerulonephropathy and a proven T-cell malignant lymphoma. The implications are discussed with reference to the literature.

Humans↗

Mycosis fungoides with spontaneously regressing CD30-positive tumorous lesions.

Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma. We report a case in which identical T-cell clones were identified in both patch and tumour stage lesions and in which the tumorous deposits, containing CD30-positive cells, regressed spontaneously. We discuss the differential diagnosis of the tumorous lesions and the spectrum of CD30-positive proliferative T-cell disorders.

Adult↗

VM 26 in advanced mycosis fungoides.

Five consecutive patients with progressive extracutaneous stage IV mycosis fungoides (MF) were treated with VM 25, 100 mg/m2 i.v., for at least 3 cycles. All patients had been extensively pretreated and in particular with vinca alkaloids. Two partial responses of 5 and 9+ months duration and 1 minimal response of 5 months duration were obtained. Transient myelosuppression was encountered in all patients. These preliminary results should encourage further exploration of VM 26, even in less advanced MF.

Adult↗

Long-term remission of recalcitrant tumour-stage mycosis fungoides following chemotherapy with pegylated liposomal doxorubicin.

Advanced stage mycosis fungoides (MF) generally has a poor prognosis, and currently there is no standard treatment available. Here we report the case of a young woman with recalcitrant tumour-stage MF (T3, stage IIb) whose disease was unresponsive to several therapeutic modalities, but who has showed sustained clinical response to pegylated liposomal doxorubucin. No severe infectious complications have been observed. The use of this drug in tumour-stage MF should be investigated further.

Adult↗

The pleomorphic cells of advanced mycosis fungoides. An ultrastructural study.

Five patients with advanced disseminated mycosis fungoides were studied, using light and electron microscopy. All had lesions with large pleomorphic cells by light microscopy. In three of the patients, nonlymphoid tissue was studied, including pericardium, epididymis, and small intestine. All areas selected for ultrastructural study showed large pleomorphic cells. We conclude that these represent poorly differentiated lymphoid cells derived from Sézary lymphocytes. This is based on nuclear and organelle similarities between the Sézary and pleomorphic cells, as well as the demonstration of intermediate forms.

Cell Nucleus↗

Balanced terminal chromosome translocations develop in EBV-derived, but non-immortal cell lines from patients with mycosis fungoides.

Five lymphoblastoid cell lines established from patients with mycosis fungoides were followed during long-term culture. They all expressed the Epstein-Barr virus nuclear antigen. Although the established B-cell lines initially showed a normal karyotype, metaphases with balanced terminal translocations gradually occurred. These chromosome abnormalities occurred simultaneously with decreased cell proliferation leading in all cases to termination of the cell lines. This contrasts the usual finding that Epstein-Barr virus established B-lymphoblastoid cell lines are immortal.

B-Lymphocytes↗

Mycosis fungoides with multiple oral mucosal lesions. A case report.

Mycosis fungoides is a cutaneous T-cell lymphoma which in its later stages affects extracutaneous sites, including the oral mucosa. A case is presented in a 69-year-old male in which oral involvement was widespread and rapidly progressive. Although gingival involvement, as seen in this patient, is relatively rare, it is useful for the periodontist to be aware of the oral manifestations of this disease since cutaneous lesions would most likely be covered if the patient were to present for consultation on an ambulatory basis.

Aged↗

Normalized light reactions in mycosis fungoides patients after complete remission of skin lesions.

Eleven mycosis fungoides patients were phototested on apparently normal skin before treatment with mechlorethamine, topically in 8 patients and with methoxsypsoralen followed by longwave ultraviolet light (PUVA) in 3 patients. Abnormal photosensitivity to UVB was seen before treatment in 4, to UVA in 4, and to visible light in one patient. The abnormal photosensitivity to all wavelength regions was normalized after complete remission of the cutaneous lesions. Two of the PUVA-treated patients demonstrated that special care should be taken during the initial phase of this treatment because of the light sensitivity, especially on lesional skin.

Aged↗

Mucinosis of the mammary areolae and mycosis fungoides.

We present a patient with cutaneous mucinosis of the mammary areolae who developed multiple erythematous macules with clearly defined, geographical edges 1 year after diagnosis. These lesions demonstrated mycosis fungoides on histology. We discuss a possible association between the mucinosis and the mycosis fungoides.

Adult↗