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11/47. Enterococcal eschars in cutaneous T-cell lymphoma tumors: a distinct clinical entity.

    Cutaneous T-cell lymphomas (CTCL) are extranodal non-Hodgkin's T-cell lymphomas that present in the skin, the most common form being mycosis fungoides. The progression of disease is associated with acquired immunodeficiency and increased susceptibility to infections. Ten CTCL patients presented with dark brown to black eschars overlying ulcerated tumors that cultured positive for enterococcus and healed with appropriate antibiotic therapy. Enterococcal infections in CTCL tumors may be recognized as a distinct clinical entity requiring specific intervention.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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12/47. Granulomatous slack skin or granulomatous mycosis fungoides -- a case report. Complete response to percutaneous radiation and interferon alpha.

    A 46-year-old man presented with circumscribed inflammatory poikilodermatic lesions of loose skin on the upper arm. Histologic examination disclosed a heavy lymphocytic infiltrate of the whole dermis and the upper part of the subcutaneous fat tissue with a predominant T-helper phenotype and about 10% of Mac 387-positive macrophages and some scattered multinucleated giant cells. Lymphocytic cells were aligned along the epidermal basement membrane and showed focal epidermotropism. In part these cells had multilobular nuclei. The diagnosis of granulomatous mycosis fungoides versus early granulomatous slack skin was made. The patient was treated with a combination of radiotherapy (total dose 36 Gy) and interferon-alpha as a maintenance treatment which resulted in complete remission and disease-free survival of 27 months up to now.
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ranking = 5
keywords = mycosis fungoides, fungoides, mycosis
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13/47. A case of Sezary's syndrome associated with granulomatous lesions, myelodysplastic syndrome and transformation into CD30-positive large-cell pleomorphic lymphoma.

    Sezary's syndrome (SS) is a leukaemic variant of mycosis fungoides, a cutaneous T-cell lymphoma showing distinct clinical, histological, immunological, and genotypic features. We report a 10-year follow-up of a patient with SS exhibiting unusual features such as granulomatous skin lesions, transformation to a CD30-positive large-cell pleomorphic T-cell lymphoma, and development of myelodysplastic syndrome and review the cases of SS reported in the literature with these unusual and rare complications.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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14/47. Long-term remission after allogeneic hematopoietic stem cell transplantation for refractory cutaneous T-cell lymphoma.

    BACKGROUND: Allogeneic hematopoietic stem cell transplantation has proved to be an effective therapeutic option in various hematologic neoplastic disorders. Because patients with advanced cutaneous T-cell lymphoma have a poor prognosis, with minimal possibilities of sustained remission, we studied the therapeutic potential of hematopoietic stem cell transplantation. OBSERVATIONS: Three young patients with refractory tumor stage mycosis fungoides underwent allogeneic HLA-matched sibling transplantation with combined marrow and CD34-enriched peripheral blood stem cell transplantation after cytoreductive chemotherapy and total-body irradiation. Complete and sustained clinical and histologic remission was achieved in 2 patients, and both remain disease free 4(1/2) years and 15 months later. One patient was in complete remission for 9 months, followed by limited cutaneous recurrence. Mild graft-vs-host disease and graft-vs-tumor effect have contained the recurring disease as a low-grade process. CONCLUSIONS: Allogeneic hematopoietic stem cell transplantation has the potential for sustained remission and the possibility of cure for young patients with advanced and recalcitrant cutaneous T-cell lymphoma. Even in the absence of complete remission, an allogeneic graft-vs-tumor effect may provide an immune mechanism to control the malignant T-cell process and alter the natural history of disease.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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15/47. Common clonal T-cell origin in a patient with T-prolymphocytic leukaemia and associated cutaneous T-cell lymphomas.

    An unusual course was observed in a patient with indolent T-prolymphocytic leukaemia (T-PLL) who subsequently developed mycosis fungoides (Mf), lymphomatoid papulosis (LyP) and cutaneous CD30 anaplastic large cell lymphoma (ALCL). polymerase chain reaction analysis demonstrated identical monoclonal T-cell receptor-beta and -gamma gene rearrangements in all the different clinical entities. Furthermore, cytogenetic studies revealed the same aberrant clone with trisomy of chromosome 8 in T-PLL and ALCL cells. This unique observation suggests that in T-PLL, the leukaemic cells might undergo secondary transformation, subsequently resulting in different phenotypes of cutaneous T-cell lymphoma.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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16/47. Mycosis on mycosis fungoides: zoophilic dermatophytosis selectively superimposed on pre-existing cutaneous T-cell lymphoma (mycosis fungoides) plaques.

    We report a case of tinea corporis caused by a cattle-derived strain of trichophyton mentagrophytes in a 44-year-old male affected by cutaneous T-cell lymphoma (CTCL, so-called mycosis fungoides). Fungal colonization of glabrous skin was strictly confined within pre-existing lymphomatous plaques. Either oral itraconazole or griseofulvin, or topical terbinafine were ineffective until the patient, who was treated with systemic retinoids and interferon-alpha for his CTCL, was shifted from leucocyte to lymphoblastoid interferon. The hypothesis that a local immunodisturbance could be responsible for the selective superimposition of tinea on CTCL lesions ('mycosis on mycosis'), and that such an immunodisturbance could be partially corrected by the interferon switch is discussed.
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ranking = 9.1557112907897
keywords = mycosis fungoides, fungoides, mycosis
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17/47. Treatment of advanced mycosis fungoides by allogeneic stem-cell transplantation with a nonmyeloablative regimen.

    Summary:Given the poor prognosis of patients with advanced cutaneous T-cell lymphoma and the high transplant-related mortality associated with conventional allogeneic bone marrow transplantation, we performed nonmyeloablative transplantation of allogeneic stem cells (ASCT) from HLA-identical siblings in three patients with this disease. All patients achieved full donor engraftment, clearance of clonal T cells leading to durable complete remissions but experienced high incidence of infections, which proved fatal in one case. These results suggest that nonmyeloablative ASCT is a novel and potentially curative therapy for patients with advanced T-cell lymphomas who have a histocompatible sibling.
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ranking = 4
keywords = mycosis fungoides, fungoides, mycosis
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18/47. Granulomatous slack skin disease--disease features and response to pentostatin.

    Granulomatous slack skin disease (GSSD) is a rare condition characterized clinically by redundant skin folds, which show a predilection towards flexural areas, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibres. The disease is often indolent, although rapid progression and transformation have been described. There is much debate as to whether this condition is a subset of mycosis fungoides or a separate disease entity in itself. We describe a case of GSSD with unique manifestations including granulomatous bone marrow involvement and hypercalcaemia. The patient has twice achieved a good response to pentostatin after failure of combination chemotherapy. This is the first report of the successful use of the purine analogue pentostatin in the management of GSSD.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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19/47. dermatomyositis associated with angiotropic lymphoma.

    We describe a 64-year-old man with dermatomyositis sine myositis who presented with unusual cutaneous plaques and nodules. Initial investigations failed to reveal evidence of malignancy. Eighteen months later he became systemically unwell and repeat biopsies from the nodules confirmed angiotropic T-cell lymphoma. Cutaneous lymphoma is rarely reported with dermatomyositis and in the previously reported cases, mycosis fungoides was the variant seen. This is the first report of angiotropic T-cell lymphoma associated with dermatomyositis.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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20/47. Syringolymphoid hyperplasia with alopecia--a syringotropic cutaneous T-cell lymphoma?

    Report on a 44-year-old male patient who developed a solitary plaque on the trunk. Histologically there is hypertrophy of adnexal duct structures and a lymphoid infiltrate with a T helper phenotype. The term 'syringotropic variant of mycosis fungoides' is proposed for this distinct disorder.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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