1/167. 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. ( info) |
2/167. 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. ( info) |
3/167. sezary syndrome in a young man with severe atopic dermatitis. sezary syndrome (SS) is a rare cutaneous T-cell lymphoma. SS usually develops de novo. We describe a 23-year-old man with a proven history of severe atopic dermatitis since childhood, who developed SS. This case contributes to the discussion about the possibility of a relationship between inflammatory dermatitis, atopy and subsequent SS. We provide criteria that should be fulfilled to define such an association. ( info) |
4/167. 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. ( info) |
5/167. 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. ( info) |
6/167. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) following treatment with deoxycoformycin in a patient with cutaneous T-cell lymphoma (sezary syndrome): A case report. We present a case of a patient who developed all manifestations of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) acutely following treatment of cutaneous T-cell lymphoma (CTCL, sezary syndrome) with deoxycoformycin (pentostatin). Symptoms and signs included severe thrombocytopenia and microangiopathic hemolytic anemia; hallucinations, confusion and disorientation; oliguric acute renal failure requiring hemodialysis; and fever. No other etiology for these symptoms and signs was present. Complete recovery followed treatment for one month with plasma exchange and glucocorticoids. During the succeeding 20 months she has remained well and her CTCL remains stable on no further treatment. This case and two previously published cases suggest that acute and severe TTP-HUS may be a dose-dependent toxicity of deoxycoformycin (pentostatin). ( info) |
7/167. Characterization of a canine long-term T cell line (DLC 01) established from a dog with sezary syndrome and producing retroviral particles. The canine DLC 01 cell line derives from a lymph node of a dog with sezary syndrome. The DLC 01 cell phenotype is CD4-, CD8 , CD45 , DQ , similar to that of original cells after treatment with dimethylsulfoxide or phorbol myristate. Canine cutaneous T cell lymphoma are usually CD4-, CD8 in contrast to their human counterparts which are CD4 , CD8-. Therefore, the DLC 01 cell line appears to be a unique model to study the mechanism of all surface molecule expression in vitro. Viral particles with retrovirus type-C morphology were found in ultrathin sections of DLC 01 cell pellets. Retroviral particles are spontaneously produced after the 50th cell passage or after induction with 0.5% dimethylsulfoxide. This is the first description of a dog lymphoid cell line spontaneously growing and producing a retrovirus. It was found to share several features in common with feline and murine leukemia viruses. ( info) |
8/167. 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. ( info) |
| 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. ( info) |
10/167. Seropositive polyarthritis and skin manifestations in T-prolymphocytic leukemia/Sezary cell leukemia variant. Sezary cell leukemia (SCL) is a rare T cell neoplasia that has been suggested to be a variant of T-prolymphocytic leukemia (T-PLL). Both disorders have an aggressive clinical course, lymphocytosis with characteristic morphology, lymphadenopathy, hepatomegaly, characteristic cytogenetic abnormalities and mature T cell phenotypes. Skin lesions, however, are mainly found in T-PLL. We describe a patient with T-PLL/SCL, who atypically presented with severe seropositive polyarthritis and skin lesions, responding to treatment with human CD52 antibody, CAMPATH-1H and pentostatin. Meningeal leukemia and an assumed myocardial infiltration subsequently developed. Polyarthritis is common in T large granular lymphocyte leukemia and adult T cell lymphoma-leukemia, but both entities could be ruled out in the present case. In rheumatoid arthritis, an expansion of CD4 and/or CD8 T lymphocytes is well documented and this phenomenon is believed to be of pathogenetic importance. We speculate that the T cell clone in the present case had special homing properties or cytokine effects resulting in synovitis. ( info) |