1/46. Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma.PURPOSE: To determine the frequency of ophthalmic abnormalities in patients with cutaneous T-cell lymphoma (mycosis fungoides and sezary syndrome) and T-cell lymphoma involving the skin and to describe the clinical course of the disease with selected examples. methods: A computerized diagnostic retrieval system was used to identify all patients with T-cell lymphoma involving the skin who were examined at the Mayo Clinic (Rochester, minnesota) between January 1, 1976 and December 31, 1990. The medical records of affected patients were reviewed. RESULTS: During the 15-year interval from 1976 through 1990, cutaneous T-cell lymphoma was diagnosed in 2,155 patients. Of these 2,155 patients, 42 (1.95%; 26 male and 16 female) had at least 1 ophthalmic abnormality attributable to the disease. The diagnoses in these 42 patients were mycosis fungoides in 19, clinical variants of T-cell lymphoma of the skin (most commonly, peripheral T-cell lymphoma) in 11, and sezary syndrome in 12. Cicatricial eyelid ectropion was the most common finding, affecting 17 (40.4%) of the 42 patients. Thirty-seven patients had findings that, although probably not a direct consequence of cutaneous T-cell lymphoma, have been cataloged in previous studies. CONCLUSION: Although ophthalmic abnormalities in patients with cutaneous T-cell lymphoma are relatively uncommon, the manifestations of the disease are diverse and frequently difficult to treat.- - - - - - - - - - ranking = 1keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
2/46. Remission of refractory sezary syndrome after bone marrow transplantation from a matched unrelated donor.sezary syndrome is a leukemic variant of mycosis fungoides (MF)/cutaneous T-cell lymphoma (CTCL). bone marrow transplantation (BMT) from a matched unrelated donor was performed in a 22-year-old woman with a 10-year history of sezary syndrome who had failed treatment with corticosteroids, methotrexate, photochemotherapy, photopheresis, hydroxyurea, interferon-alpha, and cladarabine. At the time of BMT, she had persistent erythrodermic skin disease, adenopathy, circulating Sezary cells and bone marrow (BM) involvement. The patient underwent BMT from a 6/6 HLA-matched unrelated male donor in August 1996. A BM biopsy obtained on day 30 after BMT showed no evidence of lymphoma and complete male donor engraftment. Her skin lesions resolved within 100 days after transplant. Complete staging studies, including T-cell receptor gene rearrangement studies performed at 36 months post-BMT, showed no evidence of recurrent sezary syndrome. This represents her first durable remission since the initial diagnosis more than 12 years ago. To our knowledge, this is the first patient with refractory sezary syndrome who has been successfully treated with allogeneic unrelated donor BMT. Our results indicate that this modality may be effective in inducing remission in refractory MF/CTCL, including sezary syndrome.- - - - - - - - - - ranking = 0.5keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
3/46. Large cell transformation of sezary syndrome. A conventional and molecular cytogenetic study.Hyperdiploidy sometimes is found in mycosis fungoides-sezary syndrome, but its diagnostic significance remains undefined. We report an unusual case of sezary syndrome manifesting with leukemic large cell transformation. Conventional karyotypic analysis showed the presence of a near-tetraploid neoplastic clone. With dual-color cytometric analysis, we showed that the large Sezary cells were near-tetraploid with a dna index of 1.86, thereby demonstrating a direct relationship between cell size and ploidy. comparative genomic hybridization further showed chromosomal imbalances that were not revealed on conventional karyotyping. Our findings suggest that hyperdiploidy may be a marker of large cell transformation, so that when this karyotypic abnormality is found in mycosis fungoides-sezary syndrome, a search for such a complication is indicated.- - - - - - - - - - ranking = 1keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
4/46. Non-Hodgkin's lymphoma involving the vulva.Non-Hodgkin's lymphomas (NHL) involving the vulva are rare. We report the clinicopathologic and immunohistochemical findings of six cases of vulvar NHL. The age of the patients ranged from 43 to 71 years (mean 60 years), and 5 presented with a vulvar mass. Two patients had neoplasms localized to the vulva, and two patients had a history of NHL that secondarily involved the vulva; in another patient the stage was unknown, and the sixth patient had stage IVA mycosis fungoides/sezary syndrome involving the vulva. Each tumor was classified according to the revised European-American classification of lymphoid neoplasms: four were diffuse large B-cell lymphoma, one was peripheral T-cell lymphoma, and one was mycosis fungoides/sezary syndrome. Two patients were treated with chemotherapy and radiotherapy, one patient received chemotherapy and phototherapy, one patient was treated with chemotherapy, and in two patients the treatment is unknown. Clinical follow-up, available for 4 cases, ranged from 7 months to 5 years. One patient with low-stage NHL responded to therapy, but relapsed and died of disease 2 years later. Two patients with generalized NHL that secondarily involved the vulva died of disease 7 months and 5 years, respectively, after the diagnosis of vulvar involvement was established. The patient with mycosis fungoides/sezary syndrome is alive with disease at 4 years. The three patients in this study who died and our review of the literature indicate that NHL involving the vulva is usually an aggressive disease.- - - - - - - - - - ranking = 1.5keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
5/46. sezary syndrome in a Malay--case report and literature review.sezary syndrome is a rare form of primary cutaneous T cell lymphoma. It is a distinct systemic variant of mycosis fungoides, marked by erythroderma, lymphadenopathy and circulating cerebriform lymphocytes in the peripheral blood. We report a case of sezary syndrome in a 61-year-old Malay man with a five-year history of indurated plaques, ulcers and tumours on the head and trunk, with characteristic findings on physical examination, skin biopsy, electron microscopy, immunophenotyping and peripheral blood film. A literature review on sezary syndrome is presented.- - - - - - - - - - ranking = 0.5keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
6/46. Cutaneous T-cell lymphoma in a cardiac transplant recipient.mycosis fungoides, an uncommon form of cutaneous T-cell lymphoma, arises in the skin and frequently progresses to generalized lymphadenopathy Although the cause of cutaneous T-cell lymphoma is unknown, chronic immunosuppression may play a role. A few cases have been reported in renal transplant recipients; however, ours appears to be the 1st report of cutaneous T-cell lymphoma in a cardiac transplant recipient. In our patient, cutaneous manifestations of the disease were noted less than 1 year after transplantation. Seven years after transplantation, sezary syndrome, a variant form of mycosis fungoides, was diagnosed by tissue biopsy and flow cytometry analysis. photopheresis improved symptoms but was not well tolerated because of hemodynamic sequelae. Psoralen and ultraviolet A therapy also improved the patient's skin condition, but a generalized lymphadenopathy developed. The maintenance immunosuppressive regimen was changed from cyclosporine (3 mg/kg/day) and azathioprine to cyclosporine (1.5 mg/kg/day) and cyclophosphamide. Although effective in the short-term, the results of this therapeutic strategy could not be fully evaluated because the patient died of acute myocardial infarction.- - - - - - - - - - ranking = 0.60175787937106keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
7/46. Bilateral breast involvement in sezary syndrome.Cutaneous T-cell lymphoma is a term used for mycosis fungoides and sezary syndrome, the distinct clinical entities where the skin is the primary organ of involvement. sezary syndrome is the leukemic variant of mycosis fungoides, presenting with generalized erythroderma, lymphadenopathy, and atypical cells (the Sezary cells) in the peripheral blood and bone marrow. The dissemination of cutaneous T-cell lymphoma may occur with no exception of the organs; however, no prior report exists about the sezary syndrome secondarily involving the breasts. We report the clinical and radiological findings of bilateral breast involvement in a case of sezary syndrome.- - - - - - - - - - ranking = 1keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
8/46. sezary syndrome. A malignant leukemic reticuloendotheliosis.review of the literature and the study of a case associated with a malignant lymphoma strongly suggests that the sezary syndrome is merely a clinical variant of mycosis fungoides. Additional evidence is presented that the lymphadenopathy of the sezary syndrome is not always of a benign nature, as originally indicated in the literature. Both the sezary syndrome and mycosis fungoides share the unpredictability of the neoplastic processes of the reticular system which are likely to involve simultaneously or successively, the skin and the lymph-hematopoietic system, as well as the ability of the cell involved in the process to invade the blood stream, giving rise to the leukemic variety of the malignant reticulosis.- - - - - - - - - - ranking = 1keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
9/46. 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.- - - - - - - - - - ranking = 0.5keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
10/46. 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.- - - - - - - - - - ranking = 0.5keywords = mycosis fungoides, fungoides, mycosis (Clic here for more details about this article) |
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