1/23. A case of natural killer/T cell lymphoma of the subcutis resembling subcutaneous panniculitis-like T cell lymphoma.A case of nasal type natural killer (NK)/T cell lymphoma of the subcutis showing clinical and morphological features that resemble subcutaneous panniculitis-like T cell lymphoma (SPTCL) is presented. A 73-year-old man presented with swelling of the left arm and was diagnosed with panniculitis by a dermatologist. It was concluded from a skin biopsy specimen that the patient had non-Hodgkin's lymphoma of the large cell, NK/T cell type because the neoplastic cells showed polyclonal CD3 immunoreactivity. Treatment with interferon-gamma was initiated, but the patient died of disseminated intravascular coagulation and multiple organ failure 2 months after the initial symptoms appeared. However, involvement of additional organs by the lymphoma was not apparent clinically. An autopsy was not performed. A routinely stained section of the biopsy skin specimen revealed massive necrosis of the subcutaneous fat, karyorrhexis admixed with reactive histiocytes, and large atypical lymphoid cells. Immunoreactivity for polyclonal CD3 was present in the perinuclear region, but absent in the neoplastic cell membranes. CD56, CD45RO (UCHL-1), CD43 (MT1), CD45 (leukocyte common antigen), and the cytotoxic molecules perforin, granzyme B and TIA-1 were positive, but CD20 (L26), CD4, CD8, and betaF1 were negative. Epstein-Barr virus (EBV) mRNA was detected in the nuclei of neoplastic cells by in situ hybridization. Subcutaneous panniculitis-like T cell lymphoma is reported to be an EBV-negative, clonal T cell neoplasm. Although this case showed clinical and morphological features that resembled SPTCL, perinuclear polyclonal CD3 staining and membranous CD56 reactivity seen in neoplastic cells were suggestive of NK cells. Furthermore, the neoplastic cells were positive for EBV. This case is considered to be a NK/T cell lymphoma of the subcutis resembling SPTCL. It is believed that it is important to recognize such a tumor because patients may undergo a fulminant clinical course, despite the tumor being localized in the subcutaneous adipose tissue.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
2/23. L-asparaginase induced complete remission in Epstein-Barr virus positive, multidrug resistant, cutaneous T-cell lymphoma.A 30-year-old man was admitted to our hospital with subcutaneous tumors and a high fever. Based on biomicroscopic findings of the tumor, the patient was diagnosed as having diffuse, medium, well-differentiated malignant lymphoma. Immunochemical analysis showed that CD3, CD4, CD25, and TCR beta were positive, and in situ hybridization revealed Epstein-Barr virus-encoded small RNAs in the nuclei of the lymphoma cells. Despite the patient's resistance to multidrug therapy, complete remission was achieved using L-asparaginase. This case is unique because of its peculiar clinical course and a possible association with the Epstein-Barr virus. L-asparaginase may be an important treatment in other patients who exhibit some of these characteristics.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
3/23. T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases.OBJECTIVE: Lymphomas of the eye and its adnexa are frequently of B lineage. This study aims to characterize the clinical and histopathologic features of the rare non-B-cell non-Hodgkin lymphomas (NHL) of these locations. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Seven cases of T- and T/NK-cell lymphomas involving the ocular and ocular adnexal tissues. methods: A morphologic, immunohistochemical, and molecular analysis (polymerase chain reaction) of each of the tumors was undertaken. The lesions were classified according to the Revised European-American lymphoma (REAL) classification. The clinical and follow-up data were collected. RESULTS: The patients included four women and three men ranging in age from 32 to 88 years (mean, 63 years). The presenting ophthalmic symptoms varied from a small nodule on the upper eyelid and conjunctival swellings to dramatic loss of vision associated with gross protrusion of the globe. Five of the cases presented were secondary manifestations of a systemic lymphoma in ocular tissues; two cases represented primary disease. Three cases were "peripheral T-cell lymphomas (PTCL), unspecified" with positivity for CD3, CD8, and betaF1 and negativity for CD56. Two cases were CD3 , CD30 , and CD56- and were classified as "anaplastic large-cell lymphomas of T-cell type" (T-ALCL). The remaining two cases showed an immunophenotype of CD3 , CD56 , and betaF1- and proved to contain Epstein-Barr virus (EBV) by in situ hybridization, consistent with "T/natural killer (NK)-cell lymphoma of nasal type." Clonal T-cell populations were shown in all three of the PTCLs by Southern blot (n = 1) and polymerase chain reaction (n = 2) for the T-cell receptor gamma and beta genes in one case of ALCL but not in the T/NK-cell lymphomas. Five patients died within 2 years; only two patients (one primary PTCL and one cutaneous T-ALCL) were disease free at 4 and 5 years' follow-up, respectively. CONCLUSION: This study shows that a heterogeneous group of T-cell lymphomas can involve the eye and its adnexal tissue. Most T-cell neoplasms are secondary manifestations of systemic disease and carry a poor prognosis. These findings, in conjunction with published data on ocular B-NHL, also indicate that immunophenotypic differentiation between T- and B-NHL in these locations is of clinical importance.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
4/23. Subcutaneous panniculitis by Epstein-Barr virus-infected natural killer (NK) cell proliferation terminating in aggressive subcutaneous NK cell lymphoma.We describe here a case involving a patient presenting initially with subcutaneous panniculitis, which developed after 12 years into aggressive subcutaneous natural killer (NK) cell lymphoma with peripheral blood involvement and hemophagocytosis. The surface marker of lymphoid cells in peripheral blood was CD2 3-7 8-16 56 . skin biopsies were taken in May 1986 and June 1998. The initial biopsy revealed a diffuse proliferation of atypical lymphoid cells in the subcutaneous tissue with panniculitis, while the second biopsy revealed the presence of large lymphoid cells in the subcutaneous tissue with necrotic changes, consistent with a diagnosis of malignant lymphoma (diffuse pleomorphic type). The lymphoid cells from these two specimens were positive for CD56 and such cytotoxic molecules as T-cell intracellular antigen-1 (TIA-1), granzyme B, and, interestingly, also positive for Epstein-Barr (EB) virus by in situ hybridization. This suggests that chronic EB virus infections play an important role in the early stages of tumorigenesis and in the progression of NK cell lymphoproliferative disorders.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
5/23. Aggressive Epstein-Barr virus-associated, CD8 , CD30 , CD56 , surface CD3-, natural killer (NK)-like cytotoxic T-cell lymphoma.We report an unusual case of aggressive natural killer (NK)-like cytotoxic T-cell lymphoma in a previously healthy immunocompetent West African male. He presented with a fever of unknown origin, subsequently developed erythematous skin nodules, generalized lymphadenopathy, and hepatosplenomegaly, and then died of multiple organ failure. A skin nodule and lymph node biopsy showed an infiltrate of pleomorphic atypical medium and large lymphoid cells with extensive necrosis and prominent apoptosis. Peripheral blood and ascites also harbored these cells, with cytology revealing irregular nuclear folding and basophilic cytoplasm, and some with azurophilic cytoplasmic granules. flow cytometry and immunohistochemistry demonstrated the expression of CD2, CD7, CD8, CD30, CD56, and cytoplasmic but not surface CD3. in situ hybridization demonstrated Epstein-Barr virus transcripts. A monoclonal T-cell receptor gamma chain gene rearrangement was detected by polymerase chain reaction. This is the first reported case of an NK-like T-cell lymphoma with these unusual features, making precise classification difficult. Some features suggest an NK1.1 or NKT lymphocyte origin. Because the earliest clinical manifestation was splenomegaly and abnormal liver function, the normal cellular counterpart may be a distinct subset of NK1.1 cells normally present in hepatosplenic sinusoids. This tumor disseminated early and pursued a fulminant clinical course, thus emphasizing the importance of early recognition and diagnosis.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
6/23. Cutaneous T-cell lymphoma developing in a patient on cyclosporin therapy.The use of cyclosporin in the transplant setting is associated with a small but significantly increased risk of the development of lymphoproliferative disorders. These are predominantly but not always related to Epstein-Barr virus (EBV) infection. We report a cutaneous CD30( ) T-cell lymphoma in a patient with atopic eczema during low-dose cyclosporin monotherapy. There was no evidence of EBV dna transcripts in the tumor tissue as assessed by in situ hybridization. The tumors resolved when cyclosporin therapy was stopped and have not recurred. There are a few reports of primary cutaneous lymphoma in transplant patients. This is the first report to our knowledge of cutaneous lymphoma in a patient treated with low-dose cyclosporin monotherapy. Although this finding may be coincidental, we believe this case highlights the small lymphoproliferative risk associated with cyclosporin.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
7/23. T cell receptor-gamma gene analysis of CD30 large atypical individual cells in CD30 large primary cutaneous T cell lymphomas.The hallmark of primary cutaneous CD30 large T cell lymphoma are large lymphoid tumor cells, at least 75% of which, by definition, must be positive for CD30. The relatively benign clinical course of this lymphoma type has been explained with CD30-induced apoptosis, on the assumption that expression of CD30 defines the tumor clone; however, this hypothesis has not been tested on the molecular level to date. In this study we analyzed CD30 cells in four patients with primary cutaneous CD30 large T cell lymphoma by single cell polymerase chain reaction of T cell receptor-gamma genes followed by sequencing. Here, we demonstrate that most of the large CD30 atypical cells possessed identical T cell receptor-gamma gene rearrangements, indicative of clonal proliferation. Nevertheless, polyclonally rearranged T cells were present in all CD30 samples studied. In addition, one patient showed a second clone in a separate biopsy and three of four patients showed chromosomal imbalances as revealed by comparative genomic hybridization. Taken together, our data suggest that the CD30 population in primary cutaneous CD30 large T cell lymphoma indeed contains the tumor clone, thus providing molecular support for a link between clinical course and CD30-related signaling. Importantly, however, CD30 expression does not define the tumor clone as bystander T cells, as well as occasional additional clones, are also present in this population.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
8/23. Non-cytotoxic gamma-delta peripheral T-cell lymphoma affecting the mandibular and parotidal lymph nodes and the skin.In contrast to the majority of alphabeta peripheral T cell lymphomas (PTCL), which usually originate in lymph nodes and do not express NK-associated molecules, most gammadelta PTCL express a cytotoxic phenotype and originate at extranodal sites. We report a case of a patient with a gamma-delta PTCL who presented with large mandibular and parotidal lymphadenopathy and skin lesions. CD3( )/TCR-Vdelta1 ( ) lymphoma cells did not express the cell surface (CD11b, CD11c, CD16, CD56 and CD57) and cytoplasmic granule molecules (perforin and Granzyme B) that usually characterize the cytotoxic T-cells, a phenotype that fulfils the criteria for diagnosis of a rare non-cytotoxic variant of a gammadelta T-cell lymphoma. "In situ" hybridization for Epstein-Barr virus-encoded rna and latent membrane protein-1 gave negative results. The disease had an aggressive course and was resistant to chemotherapy and the patient died 4 months after diagnosis.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
9/23. Cutaneous angiocentric T-cell lymphoma associated with Epstein-Barr virus.BACKGROUND: Two unusual cases of cutaneous angiocentric T-cell lymphoma were found to be associated with Epstein-Barr virus infection. OBJECTIVE: The objective was to study the clinical course and the response of the disease to conventional chemotherapy. methods: Histologic specimens from both patients were studied. Clonal proliferation was assessed by Southern blot hybridization. RESULTS: The disease in both patients was rapidly progressive and responded poorly to aggressive treatment. biopsy specimens showed infiltration of atypical lymphoid cells with angiocentricity and angiodestruction, which probably resulted in the observed tissue necrosis. Clonal proliferation of Epstein-Barr virus dna was detected in tissue from primary skin lesions and disseminated nasal lesions. CONCLUSION: Epstein-Barr virus-associated angiocentric T-cell lymphoma in our patients was characterized by an aggressive course and resistance to conventional chemotherapy. A search for Epstein-Barr virus and the human T-lymphotropic virus should be performed in patients with atypical features of cutaneous T-cell lymphoma.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
10/23. Nasal natural killer/T cell lymphoma with cutaneous involvement: case report and Chinese literature review reported in china mainland.Nasal natural killer (NK)/T cell lymphoma is an Epstein-Barr virus (EBV) associated lymphoma that arises in the nasal area and aggressively invades surrounding tissues. Our patient was a 48-year-old male who had had nasal obstruction and nasal discharge for 2 years and infiltrating plaques and necrosis on his nasal dorsum for three months. He developed fever and fatigue two weeks before admission. biopsy from both skin and nasal mucosa revealed atypical medium-sized tumor cells infiltrating into the dermis. Immunohistochemical studies revealed that the tumor cells were UCHL-1, cytoplasmic CD3, CD56, TIA-1, and granzyme B positive, and CD8 and CD20 negative. in situ hybridization for EBV-dna was positive. Clonal TCRb and TCRg gene rearrangement were negative. The patient was treated with cyclophosphamide, vincristine, and prednisone (COP) and with local radiotherapy, but he died 20 days later. We reviewed the cases of nasal NK/T cell lymphoma reported in mainland china in the Chinese literature during the last 5 years.- - - - - - - - - - ranking = 1keywords = hybridization (Clic here for more details about this article) |
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