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1/34. Cutaneous graft-versus-host-like reaction in systemic T-cell lymphoma.

    We report a case of systemic T-cell lymphoma with cutaneous lesions showing histological features of a cutaneous graft-versus-host-like-reaction. histology from liver, lymph node and bone marrow showed a malignant T-cell infiltrate. T-cell receptor gene rearrangement studies confirmed the diagnosis. A cutaneous graft-versus-host-like reaction has been reported with disseminated malignancy and one case has been reported with systemic lymphoma. Graft-versus-host disease normally occurs when lymphocytes from an immunocompetent donor are introduced into a histo-incompatible recipient who is incapable of rejecting them. In our patient a similar reaction may have occurred if the lymphoma was composed of cytotoxic cells or if a cell-mediated immune response against the malignant T-cells cross-reacted with epidermal keratinocytes. Alternatively the malignant T-cells could have been functionally active and induced a lichenoid reaction in the skin.
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2/34. An unusual cutaneous T cell lymphoma presenting as leonine facies.

    We report an unusual case of a cutaneous T cell lymphoma with the clinical picture of leonine facies as the only skin symptom appearing during the first years of the disease. Small atypical lymphocytes with partly pleomorphic, partly indented cerebriform nuclei are present in the facial skin as well as in the peripheral blood and in the bone marrow. The lymphoma shows an indolent clinical behaviour without rapid progression of the disease. It shares features with both pleomorphic small/medium-sized T cell lymphoma and mycosis fungoides, but cannot be classified according to the criteria of the European Organization for research and Treatment of Cancer (EORTC) classification for Primary Cutaneous Lymphomas. At present the lymphoma has to be ranged among the group of so-called unspecified peripheral T cell lymphomas according to the proposal of the International lymphoma Study Group.
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3/34. Co-existence of cutaneous T-cell lymphoma and B hairy cell leukemia.

    A primary cutaneous form of peripheral T-cell lymphoma (PTCL) and a low grade B-cell non-Hodgkin's lymphoma that was classified as a variant of hairy cell leukemia (HCL) were simultaneously diagnosed in a 79-year-old woman by both phenotypic and genotypic analyses. The coexistence of a T- and B-cell lymphoma in the same patient is rare, and, to our knowledge, this particular association has not been previously described. The patient was referred to our Department for evaluation of multiple cutaneous itchy, reddish plaques; laboratory analyses disclosed a lymphocytosis, that presented 6 years earlier. A bone marrow aspirate showed a 50% B-cell interstitial infiltrate, while a skin biopsy surprisingly revealed a PTCL. Clonality of both neoplastic processes was assessed by Southern blot analysis. The indolent clinical course of the cutaneous disease, and the low and stable number of circulating neoplastic T cells supported the diagnosis of a mycosis fungoides (MF)-like PTCL. Possible oncogenic events and/or putative underlying viral infections which could have played a role in the occurrence of B- and T-cell non-Hodgkin's lymphomas in the same patient are discussed.
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4/34. Effective high-dose chemotherapy combined with CD34 -selected autologous peripheral blood stem cell transplantation in a patient with cutaneous CD30-negative large T cell lymphoma.

    Generalized multiple cutaneous tumors developed in a 60-year-old Japanese man. skin biopsy revealed atypical large T lymphocytes infiltrating the dermis. CD30 staining was negative in the tumor cells. The diagnosis of CD30-negative cutaneous large T cell lymphoma was made. Axial and inguinal lymphadenopathy was present, but there was no evidence of bone marrow involvement. Seven cycles of chemotherapy and local electron beam irradiation were administered and complete remission (CR) was attained. As CD30-negative cutaneous large T cell lymphoma has a poor prognosis despite intensive chemotherapy, high-dose chemotherapy followed by CD34 -selected autologous peripheral blood stem cell transplantation (CD34 -APBSCT) was prescribed. The clinical course after CD34 -selected APBSCT was complicated with CMV infection occurring twice but administration of ganciclovir resolved the symptoms. He has remained in CR for 16 months after CD34 -APBSCT. This appears to be the first case report of CD34 -APBSCT in a patient with CD30-negative cutaneous large T cell lymphoma. bone marrow transplantation (2000) 25, 1315-1317.
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5/34. Cutaneous T cell lymphoma reactive CD4 cytotoxic T lymphocyte clones display a Th1 cytokine profile and use a fas-independent pathway for specific tumor cell lysis.

    We have previously described two cytotoxic T lymphocyte clones isolated from lymphocytes infiltrating a human major histocompatibility complex class II-/class I , CD4 cutaneous T cell lymphoma. These clones displayed a CD4 CD8dim (TC5) and CD4 CD8- (TC7) phenotype and mediated a specific major histocompatibility complex class I-restricted cytotoxic activity toward Cou-LB autologous tumor cell line. Our studies were performed to elucidate the mechanism involved in T-cell-clone-mediated cytotoxicity and to determine the cytokine profile of both the lymphoma cell line and specific cytotoxic T lymphocyte clones. The results indicate that, despite surface expression of Fas receptor on Cou-LB and Fas ligand induction on TC5 and TC7 cell membranes, the CD4 cytotoxic T lymphocyte clones do not use this cytotoxic mechanism to lyse their specific target. The TC7 clone uses instead a granzyme-perforin-dependent pathway. Furthermore, quantitative analysis of Th1 and Th2 cytokine mRNA expression in the cutaneous T cell lymphoma cell line as well as in TC5 and TC7 clones indicated that, whereas the tumor cells display a Th2-type profile (interleukin-4, interleukin-6, and interleukin-10), the cytotoxic T lymphocyte clones express Th1-type cytokines (interferon-gamma, granulocyte macrophage colony stimulating factor, and interleukin-2). In addition, preincubation of the tumor-infiltrating lymphocyte clones with autologous tumor cells induced their activation and subsequent amplification of the Th1-type response. These results indicate a direct contribution of the malignant cells in the Th1/Th2 imbalance observed frequently in cutaneous T cell lymphoma patients and suggest their potential role in depressed cell-mediated immunity. Identification of CD4 Th1-type cytotoxic T lymphocyte clones, the tumor antigen they recognize, and optimization of their cytokine expression profile should be useful for the design of new immunotherapy protocols in cutaneous T cell lymphoma.
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6/34. Therapy-related myelodysplastic syndrome in a case of cutaneous adult T-cell lymphoma.

    We report a case of therapy-related myelodysplastic syndrome (t-MDS) in adult T-cell lymphoma. A 69-year-old man suffered from cutaneous adult T-cell lymphoma, which was treated with radiation to the skin and combination chemotherapy of CHOP-V-MMV and VEPA-B. After 14 months of these therapies, anemia and thrombocytopenia appeared, and bone marrow aspiration smears showed immature myeloblasts, dysplastic erythroblasts, and micromegakaryocytes. Therapy-related MDS of refractory anemia with an excess of blasts was diagnosed. Cytogenetic study of the bone marrow cells showed 5q- and additional abnormalities. Rearrangement of the MLL gene was observed in the bone marrow cells. Mutations of N-ras codons at 12,13, and 61, p53 tumor suppressor gene, and monoclonal integration of human T-lymphotrophic virus -1 provirus dna were not observed in the bone marrow cells. The patient died of pneumonia 21 months after diagnosis of cutaneous adult T-cell lymphoma.
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7/34. 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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keywords = macrophage
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8/34. Extracorporeal photochemotherapy: a case report and update.

    Extracorporeal photochemotherapy (ECP) was developed at Columbia Presbyterian Medical Center in the early 1980s for the treatment of cutaneous T-cell lymphoma (CTCL). ECP is now used primarily in the treatment of that disease at more than 100 centers worldwide. It also has been shown to be potentially effective in treating several autoimmune diseases. Most recently, it has been used in reversing solid-organ transplant rejection and graft-versus-host disease following bone-marrow transplantation. In this article, we present the case of one of the first patients treated with ECP and give an update on the current status of this therapy.
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9/34. Cutaneous dissemination of nasal NK/T-cell lymphoma with bone marrow, liver and lung involvement.

    We describe a 32-year-old Korean man with a primary nasal natural killer (NK)/T-cell lymphoma (NKTCL). Combination chemotherapy and radiotherapy resulted in initial complete remission. However, cutaneous dissemination to the trunk and proximal extremities occurred 16 months later and further investigations revealed involvement of the liver, lymph nodes, lung and bone marrow. Nasal CD56 NKTCL is mostly localized to the nasal area but also shows a predilection for other sites, such as skin. Cutaneous dissemination may be an indicator of widespread metastasis to internal organs and is consistently fatal.
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10/34. Chloroma (aleukaemic leukaemia cutis) initially diagnosed as cutaneous lymphoma.

    A 65-year-old man presented in 1997 with a nodule on his back; histology showed apparent high grade T-cell lymphoma, treated after excision with radiotherapy. He relapsed with lesions on the thigh and buttock in 1998 and was treated with CHOP chemotherapy with a complete response. Further relapse occurred in 1999 with a nodule on his thigh again; he received CNOP (doxorubicin substituted with mitozantrone). At no stage was there clinical, bone marrow or radiological evidence of extra cutaneous disease. In November 2000 he presented with widespread indurated plaques and violaceous nodules. Biopsies repeated with extensive immunohistological staining diagnosed aleukaemic leukaemia cutis. Our patient was diagnosed with cutaneous T-cell lymphoma (CTCL) on the basis of clinical and haemotoxylin & eosin appearances. The correct diagnosis was made after extensive immunohistological studies (including myeloid markers) of repeat biopsies. This case illustrates the importance of diagnostic review in atypical CTCL. There is a high incidence of progression to acute myeloid leukaemia.
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