1/27. Sezary cells with hairy projections.A case of sezary syndrome where the sezary cells showed cytoplasmic hairy projections is reported. The patient had typical exfoliative erythematous dermatitis, high white cell count, atypical lymphocytes of T-phenotype with folded nuclei and bone marrow involvement. The ultra structure study showed cerebriform nucleus and cytoplasmic projections.- - - - - - - - - - ranking = 1keywords = sezary syndrome, sezary (Clic here for more details about this article) |
2/27. sezary syndrome: a case report and a review of the molecular pathomechanism and management.sezary syndrome, one of the cutaneous T-cell lymphomas, is a rare cause of generalised exfoliative dermatitis. We report a case of sezary syndrome in a 64-year-old man who had persistent erythroderma for four years and who subsequently developed inguinal lymphadenopathy and marked leukocytosis. We review the pathomechanism and management of this rare condition.- - - - - - - - - - ranking = 6.1380185510697E-5keywords = erythroderma (Clic here for more details about this article) |
3/27. Combination treatment with extracorporeal photopheresis, interferon alfa and interleukin-2 in a patient with the sezary syndrome.Extracorporeal photopheresis is generally accepted as standard therapy for the leukaemic and erythrodermic variant of cutaneous T-cell lymphoma, the sezary syndrome (SS). Because of the limited efficacy in some patients with SS, combination therapy is often necessary. We report a new combination therapy for an intensively treated 62-year-old woman with advanced SS (T4N1BM1, stage IVb). Previous treatment with PUVA, retinoids alone and in combination with photopheresis, chlorambucil, and chemotherapy using cyclophosphamide, doxorubicin, vincristine and prednisone failed and were associated with significant side-effects. Six cycles of combination therapy with extracorporeal photopheresis, low-dose interferon alfa and interleukin-2 resulted in fading of the erythroderma and in a decrease of Sezary cells in the white blood cell count. The CD4/CD8 ratio decreased from 66 to 6 and the proportion of CD4 CD7 - cells from 47% to 11%. Only mild side-effects such as influenza-like symptoms, fever and nausea were observed. Two months after this therapy, the patient developed enlarged lymph nodes without erythroderma, and died 1 year later from the lymphoma. Combination therapy with extracorporeal photopheresis, interferon alfa and interleukin-2 might be useful in selected patients with SS.- - - - - - - - - - ranking = 0.00012276037102139keywords = erythroderma (Clic here for more details about this article) |
4/27. A case of pre-sezary syndrome preceded by hand lesions.Pre-sezary syndrome is an erythroderma with a chronic course, clinical findings of sezary syndrome, lymphocytic subepidermal band infiltration at times, and repeated cycles of circulating Sezary cells of less than 1,000 cells/mm3. Duration of the pre-existing skin diseases preceding pre-Sezary erythroderma varies from a few weeks to 20 years. Before the erythroderma develops, these patients are diagnosed with contact dermatitis, neurodermatitis, chronic dermatitis, atopic dermatitis, or asteatotic eczema. hand lesion also precedes the pre-Sezary erythroderma. This condition has been controlled by three cycles of chemotherapy consisting of vincristine, cytoxan, doxorubicin, and prednisolone. We describe a case of pre-sezary syndrome preceded by hand lesion and treated with chemotherapy.- - - - - - - - - - ranking = 0.00024552074204279keywords = erythroderma (Clic here for more details about this article) |
5/27. 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 = 6.1380185510697E-5keywords = erythroderma (Clic here for more details about this article) |
6/27. 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 = 6.1380185510697E-5keywords = erythroderma (Clic here for more details about this article) |
7/27. Sustained remission of sezary syndrome.sezary syndrome, an aggressive form of cutaneous T-cell lymphoma, is a devastating, highly symptomatic form of non-Hodgkin lymphoma. Malignant clones of mature helper CD4 T cells containing large, convoluted nuclei known as Sezary cells circulate in the blood and infiltrate the skin. Clinical features include exfoliative erythroderma, generalized lymphadenopathy, alopecia, onychodystrophy, palmoplantar hyperkeratosis, and ectropion. patients often have severe pruritus, burning sensations, pain, bleeding from excoriations, and disfigurement. Extracorporeal photopheresis, an immunomodulatory therapy, has become a primary therapy for these patients. This pheresis-based therapy uses psoralen and ultraviolet A radiation-mediated photochemotherapy to induce immune responses. The effects of extracorporeal photopheresis vary considerably. We report sustained remission (2 years) in a patient with sezary syndrome. Previously he had received extracorporeal photopheresis and interferon alfa-2b injections. He is the only one of 55 patients with sezary syndrome treated at Mayo Clinic (Rochester, minnesota, USA) to achieve sustained remission on extracorporeal photopheresis alone.- - - - - - - - - - ranking = 6.1380185510697E-5keywords = erythroderma (Clic here for more details about this article) |
8/27. Hydantoin-induced cutaneous pseudolymphoma with clinical, pathologic, and immunologic aspects of sezary syndrome.BACKGROUND--The phenytoin-induced hypersensitivity syndrome is characterized by the development of fever, rash, lymphadenopathy, and hepatitis associated with leukocytosis and eosinophilia. This article describes the unusual occurrence of a pseudo-sezary syndrome in the days following the introduction of phenytoin treatment. observation--A phenytoin-induced erythroderma developed in a 60-year-old woman the histologic, cytologic, and immunologic characteristics of an erythrodermal cutaneous T-cell lymphoma of the sezary syndrome type with lymph node involvement. The dramatic improvement after withdrawal of drug therapy and the absence of recurrence 5 years after led us to consider it as a hydantoin-induced pseudolymphoma. CONCLUSIONS--Although lymph node pseudolymphomas induced by phenytoin are well known, few cases of hydantoin-induced mycosis fungoides have been reported in the literature. We present herein the first case of a Sezary-like syndrome associated with phenytoin therapy. Such a patient must be monitored regularly because of the risk of a true malignant lymphoma developing even many years later.- - - - - - - - - - ranking = 0.00012276037102139keywords = erythroderma (Clic here for more details about this article) |
9/27. Leukoderma associated with sezary syndrome: a rare presentation.We describe a patient with sezary syndrome who was initially seen with the rare occurrence of both generalized erythroderma and extensive leukoderma of his lower limbs. Apart from the absence of melanocytes in the leukodermic skin, histopathologic features of both areas were identical, showing an infiltrate of atypical lymphocytes with epidermotropism. This form of sezary syndrome-associated leukoderma is clinically distinguishable from hypopigmented mycosis fungoides in which hypopigmented lesions are the sole manifestation of the cutaneous T-cell lymphoma.- - - - - - - - - - ranking = 6.1380185510697E-5keywords = erythroderma (Clic here for more details about this article) |
10/27. Successful treatment of chemotherapy-refractory sezary syndrome with alemtuzumab (Campath-1H).INTRODUCTION: sezary syndrome (SS) is a cutaneous T-cell lymphoma characterized by erythroderma, lymphadenopathy and circulating atypical T cells. Median survival after diagnosis is 10 yr, with chemotherapy resistance being a major problem in advanced disease. Alemtuzumab (Campath-1H) is a monoclonal antibody directed against the lymphocytic antigen CD52, expressed on B- and T-cells. Alemtuzumab is approved for relapsing chronic B-cell leukemia and seems to be active also in T-cell lymphomas such as T-cell prolymphocytic lymphoma, SS and mycosis fungiodes. CASE history: A 32-yr-old male patient presented with advanced stage, extensively pretreated SS with heavily itching erythroderma, peripheral lymphadenopathy, circulating Sezary cells and bone marrow infiltration. The disease had not responded to PUVA/interferon-alpha and progressed on chemotherapy with CHOP, 2-CDA, vinorelbine, etoposide and liposomal doxorubicin. Following treatment with alemtuzumab (30 mg i.v. three times per week for 10 wk), itching resolved rapidly and an almost complete remission was achieved within 3 months after starting this treatment. At 12-month follow up, no disease progression was present. CONCLUSION: In accordance with previous data, this single case underlines the potent activity of alemtuzumab in advanced, chemotherapy-refractory SS.- - - - - - - - - - ranking = 0.00012276037102139keywords = erythroderma (Clic here for more details about this article) |
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