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1/146. Peripheral T cell lymphoma in a patient with common variable immunodeficiency disease: case report and literature review.

    This report documents the occurrence of a peripheral T cell lymphoma arising in the bone marrow and liver of a patient with common variable immunodeficiency disease. The T cell origin of this lymphoma was demonstrated by immunohistochemical phenotyping and gene rearrangement studies and was not associated with EBV infection of the lymphoma cells. The frequency and characteristics of lymphomas complicating CVID are reviewed.
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2/146. immunoblastic lymphadenopathy-like T-cell lymphoma complicated by multiple gastrointestinal involvement.

    We report a rare case of immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma complicated by multiple gastrointestinal involvement, which appeared to be ameliorated by chemotherapy but resulted in perforative peritonitis. A 66-year-old Japanese woman who had generalized lymphadenopathy and eruptions was admitted to our hospital because of bloody stool. Colonoscopic examination revealed hemorrhagic ulcers in the terminal ileum and a saucer-like ulcer in the cecum. Gastrointestinal endoscopy revealed several ulcerative or elevated lesions in stomach and duodenum. biopsy specimens of these lesions and of a lymph node showed characteristic histological features of IBL-like T-cell lymphoma. The initial treatment with prednisolone (PSL) and cyclophosphamide (CPA) was effective. Six months after the treatment, however, she developed bloody stool again caused by multiple ulcerative lesions in the large intestine. The recurrence of the disease was determined histologically, and four courses of CPA, PSL, vinblastine sulfate and doxorubicin hydrochloride (CHOP) therapy were administered. One month after completing the CHOP therapy, she developed intestinal obstruction and then acute peritonitis resulting from perforation at an ulcer scar in the jejunum. Surgical treatment was successful, and histological examination demonstrated no lymphoma cells in the resected specimen. A gastrointestinal perforation should be recognized as a potential complication of IBL-like T-cell lymphoma, even during remission.
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3/146. gamma delta T-cell lymphoma: a clinicopathologic study of 6 cases including extrahepatosplenic type.

    We report 6 cases of gamma delta T-cell lymphoma (GDTL) among 115 peripheral T-cell lymphomas over a 12-year period. All patients exhibited extranodal lymphomas, comprising 3 hepatosplenic, 1 cutaneous, 1 intestinal, and 1 thyroidal lymphoma. Despite therapies, all but 1 patient died of disease within 16 months of diagnosis. The cytologic features of lymphoma cells in 4 of 5 cases examined were very similar: coarsely reticulated nuclear chromatin, multiple small nucleoli, abundant faintly eosinophilic granular cytoplasm, and focal angiocentric proliferation. All 6 lymphomas showed Cd3 CD4-CD8-/ phenotype. CD56 was positive in 3 cases, 1 of which was also CD16 positive. perforin and T-cell intracellular antigen-1 were positive in all 5 cases examined. Southern blot analysis revealed clonal gene rearrangements of the T-cell receptor delta-chain gene in all 5 cases examined. Based on these findings, together with a review of the literature, GDTLs seem to have several common lineage-specific features, although clinical presentation and course of GDTL are heterogeneous.
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4/146. CD30 positive (non-anaplastic) peripheral T-cell lymphoma of the thyroid gland.

    Primary non-Hodgkin's lymphoma of the thyroid gland are infrequent tumors. They almost exclusively derive from B cells of mucosa-associated lymphatic tissue and only a very small minority of them is represented by T cell lymphomas. CD30 molecule, other than in Hodgkin's and Redd-Sternberg' cells, is strictly associated with anaplastic large cell lymphoma and ALK lymphomas, the latter being identified by the monoclonal antibody ALK1. We report a case of CD30-positive non-anaplastic (ALK1-negative) peripheral T cell lymphoma of the thyroid gland and speculate on aspects concerning diagnosis and the morphologic and immunohistochemical findings.
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5/146. Revision of the diagnosis of T-zone lymphoma in the father of a patient with autoimmune lymphoproliferative syndrome type II.

    autoimmune lymphoproliferative syndrome (ALPS) is a disease of childhood characterized by typical clinical and laboratory findings. Here we describe an adult patient presenting with lymph node enlargement and splenomegaly. Pathological examination of an adenopathy supported the diagnosis of malignant T-zone lymphoma. The patient was treated accordingly. 3 years later his child was diagnosed with ALPS. Therefore the diagnosis of the father's disease was reconsidered. review of the slides and functional tests led to the diagnosis of ALPS in both father and son. ALPS should be considered as a possible differential diagnosis in adult patients presenting with rare types of T-cell lymphomas.
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6/146. Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection.

    We report three cases of nodal peripheral T-cell lymphoma (PTCL) with Reed-Sternberg-like (RS-like) cells of B-cell pheno- and/or genotype. Histologic analysis in all cases revealed diffuse nodal effacement by atypical lymphoid cells of variable size. Two of the three cases had features of angioimmunoblastic T-cell lymphoma (AILT). Large mononuclear and binucleated cells with prominent eosinophilic nucleoli and abundant cytoplasm resembling classic RS cells and mononuclear variants were scattered throughout all biopsies. The lymphoma cells in the three cases were of T-cell lineage (CD3 , CD43 , and CD45RO ). The RS-like cells from all cases were CD30 and CD15 positive. In contrast to the neoplastic T cells, the RS-like cells lacked all T-cell markers and in two cases were positive for CD20. Epstein-Barr virus (EBV) latent membrane protein 1 (LMP1) and EBER 1 (2/2) were detected in the RS-like cells in all cases. The neoplastic T cells were negative for EBV. polymerase chain reaction (PCR) analysis demonstrated clonal rearrangements of the T-cell receptor gamma chain gene in the three cases. PCR analysis of microdissected RS-like cells for immunoglobulin heavy chain gene rearrangements in cases 1 and 3 showed an oligoclonal pattern. The presence of RS-like cells in PTCL represents a diagnostic pitfall, because in one case this observation led to a misdiagnosis of Hodgkin's disease (HD). The oligoclonal expansion of EBV-infected cells may be related to underlying immunodeficiency associated with T-cell lymphomas and AILT in particular. This phenomenon may provide the basis for some cases of Hodgkin's disease after T-cell lymphomas and suggests that they are clonally unrelated neoplasms. The expression of LMP1 appears to be crucial for the immunophenotype and probably for the morphology of the RS and RS-like cells appearing in diverse lymphoid malignancies, including HD, chronic lymphocytic leukemia, and PTCL.
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7/146. 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.
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8/146. Extranodal peripheral T-cell lymphoma with angiocentric growth pattern and Epstein-Barr viral dna associated affecting paratesticular soft tissue.

    Peripheral T-cell lymphomas are uncommon, accounting for only 10% to 15% of all non-Hodgkin lymphomas and their classification has been controversial. We report a case of peripheral T-cell lymphoma with angiocentric growth pattern which presented as a paratesticular tumoral nodule in a 47-year-old-man. Formalin-fixed paraffin-embedded samples from the paratesticular tumor and non-infiltrated adjacent tissue were submitted to histological, immunohistochemical, polymerase chain reaction (PCR)-based and in situ hybridization analysis. Histopathologically, there was a lymphomatous infiltrate in the paratesticular soft tissue, composed of a variable mixture of medium-sized to large cells with large cytoplasm and irregular-shaped nuclei, together with blood vessel destruction, necrosis and karyorrhexis. Immunohistochemical study revealed a high p53 expression in neoplastic cells that showed T cytotoxic immunophenotype, failing to express the natural killer (NK)-cell antigen CD56. A monoclonal rearrangement of the T-cell receptor (TCR) gamma gene by a PCR technique was demonstrated. Type-A Epstein-Barr Virus (EBV) dna was detected by PCR-based analysis. A combined in situ hybridization and immunohistochemical study revealed that most cells labeled positive for EBV rna showed immunostaining with the CD45RO antibody. Based on the above results, the case reported was classified as extranodal peripheral T-cell lymphoma with cytotoxic phenotype and EBV associated. The present case does not fit neatly into any of the specific types of peripheral T-cell lymphomas of the REAL classification, so a diagnosis of peripheral T-cell lymphoma unspecified was made.
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9/146. Peripheral T-cell lymphoma other than angioimmunoblastic T-cell lymphoma (AILD), with follicular dendritic cells proliferation and infection of B immunoblasts with Epstein Barr virus.

    We describe a case of peripheral T-cell lymphoma, unspecified (REAL) with co-existence of follicular dendritic cell (FDC) proliferation and EB virus-infection. A 55-year-old Japanese man complained of generalized lymphadenopathy and physical examination showed systemic lymphadenopathy, and hepatosplenomegaly. HTLV-1 antibody titers and gamma-globulin level were within normal limits. Histopathologic examination of the right cervical lymph node showed peripheral T-cell lymphoma, unspecified (REAL classification). A diffuse infiltration of lymphoma cells with abundant small venules were found throughout the lymph node. There were few arborizing venules. An irregular meshwork of FDCs was found by immunostaining with DRC-1 and CD21 in the area rich in venules, but not in the area of diffuse lymphoma cell-infiltration. EBER-1 rna-in situ hybridization showed positive signal on the nuclei of mainly non-neoplastic B immunoblasts. The present case, therefore, was regarded as a rare case of peripheral T-cell lymphoma, unspecified with FDC proliferation and EB virus infection.
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10/146. Lymphoepithelioid cell lymphoma (Lennert's lymphoma) presenting as atypical granuloma annulare.

    We describe a case presenting as atypical granuloma annulare where the underlying diagnosis, confirmed by lymph node biopsy, was lymphoepithelioid cell lymphoma (Lennert's lymphoma). Lennert's lymphoma is a peripheral T-cell lymphoma which may follow a variable course and transform into an aggressive phase. Cutaneous manifestations of this condition have only rarely been reported in the literature. The presence of granulomas in the skin may have either obscured the lymphoma infiltrate or may have reflected a more generalized immune response to the underlying malignancy.
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