Cases reported "Mycosis Fungoides"

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1/24. Regression of cutaneous neoplasms following delayed-type hypersensitivity challenge reactions to microbial antigens or lymphokines.

    Induction of delayed-type hypersensitivity challenge reactions to microbial antigens at sites of neoplasms involving the skin resulted in regression of mycosis fungoides, reticulum cell sarcoma, superficial basal cell carcinoma and adenocarcinoma of the breast. Similar reactions induced by lymphokine preparations also resulted in regression of lesions of mycosis fungoides and superficial basal cell carcinoma. The role of the large monomuclear cells in the inflammatory infiltrate of the delayed hypersensitivity reaction in eliciting the tumor regression is discussed. It is proposed that these large mononuclear cells represent the effectors in a primitive surveillance mechanism for neoplastic cells.
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2/24. Polyclonal expansion of T cells with the TCR V beta type of the tumour cell in lesions of cutaneous T-cell lymphoma: evidence for possible superantigen involvement.

    The involvement of superantigens in the pathology of cutaneous T-cell lymphomas (CTCL) has been suggested before, but without unequivocal evidence for superantigen activity in the patients. Seeking evidence for superantigen activity we analysed clones and microdissected single cells isolated from the epidermis of early-stage lesions of a CTCL patient for their T-cell receptor (TCR) V beta expression and TCR V gamma gene rearrangements. The vast majority of these T cells expressed the TCR V beta family type of the tumour. From their TCR gamma gene rearrangements, however, these cells were polyclonal. The tumour cell clone accounted for about 60% of these cells, about 40% were of heterogeneous origin. This dominance of a single V beta family in the polyclonally expanded dermal T-cell populations implies superantigen activity in the CTCL lesions.
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3/24. Immunophenotypic and genetic characterization of a CD8 positive mantle cell lymphoma in a patient with concomitant mycosis fungoides.

    Mantle cell lymphoma (MCL) is immunophenotypically characterized by cell surface co-expression of CD19, CD20, CD5, IgM and FMC7. However, the concomitant presence of other antigens distinctive of a particular leukocyte subset, e.g. t-lymphocytes, is an exceptional finding in MCL. Here, the first case of a blastic MCL in leukaemic phase with aberrant expression of the T-cell associated antigen CD8 occurring in a patient with concomitant mycosis fungoides is described. Comprehensive immunophenotypic analysis showed that the MCL cells expressed the typical B-lymphocytic markers, were CD5 and CD8 positive, but did not express other T-cell proteins, such as CD2, CD3, CD4, CD7, TCRalphabeta and TCRgammadelta. The MCL cells expressed both CD8alpha and CD8beta chains indicating cell surface presence of CD8alphabeta heterodimers. Intriguingly, expression of the cytotoxic enzymes perforin and granzyme A was detected by RT-PCR. Cytogenetic and molecular genetic analysis of the lymphoma cells confirmed cyclin d1 overexpression secondary to the t(11;14)(q13;32) chromosomal translocation. Furthermore, trisomy 11, trisomy 14 and extra copies of t(11;14) translocated chromosomes were detected in sub clones of the analyzed MCL cells. Clinically, an aggressive course of disease including cerebral lymphoma involvement was noted in the reported patient. Hence, systematic studies addressing the incidence, biology and clinical behavior of this form of MCL seem to be justified in future.
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4/24. mycosis fungoides with a CD56 immunophenotype.

    We report 3 cases of mycosis fungoides (MF) with a CD56 cytotoxic immunophenotype. Each patient presented with a different clinical phenotype: one exhibited limited poikilodermatous patches (skin stage T1); one, widespread hypopigmented lesions (skin stage T2); and one, poikiloderma with a single cutaneous tumor (skin stage T3). MF was confirmed both histologically and by the presence of a T-cell receptor clone in lesional skin in all cases. CD56 and T-cell intracellular antigen-1 were expressed by the malignant lymphocytes in all patients and two expressed CD8. No sample demonstrated loss of the pan T-cell markers CD2 or CD3. None of the 3 developed systemic disease and T-cell receptor gene analysis of peripheral blood was polyclonal in all cases. Only 3 cases of CD56 MF have been reported previously, none of which exhibited tumor-stage disease. Currently, the disease in our patients appears to be behaving in a manner similar to that predicted for MF with a normal immunophenotype but the prognosis has to be guarded in view of the rarity of this subtype.
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5/24. Differential expression of CD24-related epitopes in mycosis fungoides/sezary syndrome: a potential marker for circulating Sezary cells.

    In the hematopoietic system, the B-cell associated antigen CD24 is expressed at high density on B cells, B-cell precursors, and B-cell malignancies as well as at low density on peripheral blood polymorphonuclear leukocytes. The 42-Kd sialoglycoprotein has not been previously demonstrated to be expressed on T cells, thymocytes, or T-cell malignancies. We identified three patients with mycosis fungoides/sezary syndrome that showed low density expression of the CD24-related epitope recognized by antibody BA-1 on circulating T cells. All three patients had Sezary cells by morphologic assessment and clonal T-cell populations in the peripheral blood by gene rearrangement studies. In two of these patients, indirect immunofluorescence with a panel of six anti-CD24 monoclonal antibodies demonstrated reactivity for two of six antibodies in one case and only one of six antibodies in the other. The biologic significance of CD24-related epitope expression on circulating T cells in mycosis fungoides/sezary syndrome is unclear. However, these findings suggest that differential, low density expression of CD24-related epitopes (BA-1 , OKB2-) may be a useful phenotypic marker for identifying circulating Sezary cells.
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6/24. Three cases of lymphomatoid papulosis with a CD56 immunophenotype.

    We report 3 cases of lymphomatoid papulosis (LyP) with a CD56 , cytotoxic immunophenotype. All 3 patients presented with clinical histories typical of LyP, with one patient having associated mycosis fungoides. Histologically, two cases were type A LyP and one was type B. All 3 cases demonstrated a T-cell receptor clone in lesional skin without evidence of blood involvement. The atypical lymphocytes in each of the 3 cases expressed cytotoxic granules (T-cell intracellular antigen-1 and granzyme B ) and were CD8 and CD56 . Expression of CD56 is associated with a poor prognosis in subcutaneous panniculitis-like T-cell lymphoma and blastic natural killer cell lymphoma. However, the two cases of CD56 LyP previously reported and the 3 cases in this series all appear to be pursuing an indolent course with no evidence of systemic disease.
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7/24. Study of T-cell antigen receptor gene rearrangement: a useful tool for early diagnosis of mycosis fungoides.

    A 50-year-old man was admitted to the hospital with a diagnosis of eczema. Using study of T-cell receptor gene rearrangement on skin biopsies, a diagnosis of mycosis fungoides was made, then confirmed by evolution. This observation provides the opportunity to discuss the usefulness of molecular biology for the early diagnosis of mycosis fungoides.
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8/24. Functional properties in Sezary cells with an unusual phenotype.

    The immunological and functional characteristics of Sezary cells with an unusual phenotype are reported. The clinical, histologic, and hematologic picture was typical for sezary syndrome. Studies with monoclonal antibodies showed that 80% Sezary cells had an CD3 , CD4 , CD5 , CD7-, CD8-, Leu-7 , Leu-8-, Leu-11-, OKM1- phenotype. By two-color immunofluorescence assay 80% FACS-sorted Leu-7 cells coexpressed CD4 antigen and did not express the myeloid antigen OKM1, CD8, and antigens characteristic of immature T cells. The cells had no NK activity but did display a high helper activity. Unseparated and FACS-sorted Leu-7 and Leu-7- Sezary cells did not respond to mitogens but were able to grow in the presence of exogenous IL-2. FACS sorted Leu-7- cells, cultured for 7 days in the presence of 20% IL-2, acquired the receptors for Leu-7. IL-2 and IFN-gamma production was studied in unseparated Leu-7 and Leu-7- FACS-sorted Sezary cells. IL-2 production was lower than in normal cells. The addition of PHA or PHA plus TPA led to an increase in IL-2 production. Also IFN-gamma production was marked lower than in normal controls but increased after 7-day culture in exogenous IL-2. In conclusion in this case the Sezary cells may represent a neoplastic expansion of the CD3 , CD4 , CD5 , Leu-7 , Leu-11- subpopulation which is equivalent to the 2-4% of the Leu-7 population in normal lymphocytes.
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9/24. Disseminated pagetoid reticulosis associated with mycosis fungoides: immunomorphologic study.

    We report a 77-year-old woman with erythrosquamous plaques on the legs for 10 years. Biopsies taken 4 years ago revealed a pagetoid reticulosis with a massive epidermal cell infiltrate. She has now also developed a typical tumor of mycosis fungoides. The epidermal infiltrate of the two types of lesions bore the surface membrane marker for T lymphocytes rich in both T helper and T cytotoxic-suppressor subsets (ratio, 1.8). Both lesions also showed dendritic OKT6-positive langerhans cells, and staining of the intercellular material with the DR locus of human lymphocyte antigen was positive. A dense dermal infiltrate was evident only in the mycosis fungoides lesions, and it was similar to that in the epidermis. Electron microscopy of the two lesions showed the atypical cells described in mycosis fungoides and the sezary syndrome. These observations suggest that disseminated pagetoid reticulosis probably is a variant of mycosis fungoides.
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10/24. Leu-M1 antigen expression in advanced (tumor) stage mycosis fungoides.

    The authors describe two patients with clinically and histopathologically documented advanced (tumor) stage mycosis fungoides. In each case the large, pleomorphic neoplastic cells lacked the monoclonal antibody-defined cell surface antigens commonly associated with immature and mature T-cells, i.e., T11, Leu-1, T3, T4, T6, T8, and T10, but expressed various T-cell-associated activation antigens, such as HLA-DR, Tac, and T21. Leu-M1, a monocyte-associated antigen, was not expressed by the small, cerebriform neoplastic cells in the plaque stage lesions of either patient. However, Leu-M1 was expressed by most of the large, pleomorphic neoplastic cells present in the nodular lesions of both patients. The pattern of Leu-M1 antigen expression was identical to that previously reported in the reed-sternberg cells of Hodgkin's disease. Identification of these two patients suggests using caution in the interpretation of the results of immunophenotypic analysis of cutaneous lymphoid neoplasms and that Leu-M1 should not be used as a diagnostic indicator of Hodgkin's disease or a histiocytic-derived neoplasm. These studies also suggest that Leu-M1 may be preferentially expressed on a subpopulation of activated, rapidly proliferating, and/or dedifferentiated neoplastic T-cells that proliferate in the advanced (tumor) stages of mycosis fungoides.
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