Cases reported "Lymphoma, B-Cell"

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1/159. Non-Hodgkin's lymphoma of the maxillary sinus in a patient with acquired immunodeficiency syndrome.

    Non-Hodgkin's lymphoma (NHL) is one of the most common malignancies in patients infected with human immunodeficiency virus (hiv): it occurs 25-60 times more frequently in hiv-infected patients than in the general population. This neoplasm in acquired immunodeficiency syndrome (AIDS) patients is a highly aggressive tumour with a poor prognosis and tends to develop in extranodal sites, such as the central nervous system, digestive tract and bone marrow. NHL involving the paranasal sinuses is rare in hiv-infected patients, and is likely to be confused clinically and radiographically with sinusitis; moreover, its optimal treatment is currently uncertain. We present a case of NHL involving the left maxillary sinus in a patient with AIDS. The patient was treated with systemic chemotherapy (low dose-CHOP), but the malignancy did not respond. Subsequently, he was treated with local maxillary sinus irradiation which resulted in partial regression of the neoplasm and in decrease of local symptoms.
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2/159. The blastic variant of mantle cell lymphoma arising in Waldeyer's tonsillar ring.

    We present three cases of blastic mantle cell lymphoma with an unusual initial manifestation in Waldeyer's ring with methods for differentiating it from other blastic neoplasms of the head and neck. All cases presented with a feeling of fullness in the area of the mass. Morphologically, the tumours were blastic with a high mitotic rate (three to nine per high power field). All were B-cell phenotype with coexpression of CD43. In all cases cyclin d1 and bcl-2 were positive and CD23 negative. Blastic mantle cell lymphoma occurring in Waldeyer's tonsillar ring may be mistaken for other high grade haematopoietic neoplasms. immunohistochemistry and awareness of this type of lymphoma are helpful in differentiating it from other neoplasms.
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3/159. Primary juxtaarticular soft tissue lymphoma arising in the vicinity of inflamed joints in patients with rheumatoid arthritis.

    AIMS: Primary soft tissue lymphoma is uncommon and little is known regarding its aetiology and pathogenesis. In a review of 37 soft tissue lymphomas we uncovered three cases associated with rheumatoid arthritis which we report herein. methods AND RESULTS: The clinical records and pathology of the cases are described together with the results of in situ hybridization studies with oligonucleotide probes to Epstein-Barr virus (EBV) encoded rna (EBER). All three patients were females with a long-standing history of rheumatoid arthritis ranging from 9 to 17 years. Each presented with a soft tissue mass in the vicinity of a joint affected by rheumatoid disease. All had received prior treatment with nonsteroidal anti-inflammatory drugs and one also received gold, penicillamine and intra-articular steroids to affected joints. None had received methotrexate. Histologically, the juxtaarticular soft tissue masses were all B-cell lymphomas. None were associated with EBV as determined by in situ hybridization. CONCLUSIONS: These cases document an association between rheumatoid arthritis and soft tissue lymphoma of B-cell type, arising in the vicinity of an affected joint. Chronic local immune stimulation may have played a significant role in the genesis of these lymphomas, unlike the frequently reversible and EBV-positive lymphomas that occur in rheumatoid patients on immunosuppressive therapy.
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4/159. T-cell-rich B-cell non-Hodgkin's lymphoma mimicking Hodgkin's disease.

    We report on a patient with recurrent T-cell-rich B-cell lymphoma (TCRBCL), initially misdiagnosed as a lymphocyte-rich Hodgkin's disease. This case exemplifies the diagnostic problems of TCRBCL and the need for immunophenotypic analysis to differentiate TCRBCL from Hodgkin's disease, nodular paragranuloma and peripheral T-cell lymphoma. A rather unusual aspect is the long disease-free interval between the excision of the node in and the late relapse in 1996. The significance of the abundant T-cell infiltration in this B-cell neoplasm will be discussed and the concepts concerning antitumor response will be reviewed. Based on epidemiological data and the clinical behaviour TCRBCL does not seem to represent a distinctive pathological entity.
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5/159. Composite low grade B-cell lymphomas with two immunophenotypically distinct cell populations are true biclonal lymphomas. A molecular analysis using laser capture microdissection.

    Low grade B-cell lymphomas comprise several well defined, clinically and immunophenotypically distinct disease entities. Composite lymphomas showing phenotypic characteristics of more than one of these tumor subtypes in the same site are rare, and both common and separate clonal origins of the two tumor parts have been reported for cases studied by molecular methods. We describe the detailed immunohistochemical and molecular findings in three cases with features of composite low grade B-cell non-Hodgkin's lymphoma (B-NHL). All three neoplasms contained morphologically distinct but interwoven compartments of different cell types, which exhibited discordant expression of several markers, including CD5, CD10, CD43, and cyclin d1. According to their morphology and phenotypes, they were classified as mantle cell lymphoma and follicular lymphoma (Case 1), follicular lymphoma and small lymphocytic lymphoma (Case 2), and mantle cell lymphoma and chronic lymphocytic leukemia/small lymphocytic lymphoma (Case 3). PCR analysis of dna obtained from whole tissue sections failed to reveal evidence for biclonality in any of the cases. We therefore isolated cell populations with different antigen expression patterns by laser capture microdissection and analyzed them by polymerase chain reaction amplification and sequencing of clonal immunoglobulin heavy chain gene rearrangements and oncogene rearrangements. sequence analysis revealed unrelated clonal rearrangements in each of the two tumor parts in all three cases, suggesting distinct clonal origins. In addition, Case 1 showed a bcl-2 rearrangement present only in the follicular lymphoma part. Our findings suggest that low grade B-NHL with two distinct morphological and immunophenotypic patterns in the same anatomical site are frequently biclonal. This is in keeping with current classification schemes, which recognize subtypes of low grade B-NHL as separate disease entities. Furthermore, our analysis demonstrates the power of laser capture microdissection in revealing molecular microheterogeneity in complex neoplasms.
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6/159. Kaposi's sarcoma-associated herpesvirus-positive primary effusion lymphoma arising in the subarachnoid space.

    Primary effusion lymphoma (PEL) is a rare and distinctive type of B-cell non-Hodgkin's lymphoma (NHL) that occurs primarily, although not exclusively, in patients with AIDS. It usually develops as a lymphomatous effusion in the absence of a tumor mass, characteristically contains the Kaposi's sarcoma-associated herpesvirus/human herpesvirus 8 (KSHV/HHV-8), usually also contains the Epstein-Barr virus (EBV), displays a characteristic cytomorphology bridging immunoblastic and anaplastic large cell lymphoma, often expresses an indeterminate immunophenotype, and a B-cell genotype. Thus far, PEL has been limited almost entirely to the pleural, peritoneal, and pericardial cavities. We describe a NHL occurring in a gay man with AIDS that is typical of PEL in that it arose in a body cavity or space without an associated tumor mass, displays the cytomorphology typical of PEL, is a clonal B-cell neoplasm, and contains KSHV as well as EBV. This case is singularly distinctive in that it is the first case of PEL reported to arise in the subarachnoid space. This unique case further supports the strong association between KSHV and malignant lymphoma arising in body cavities and growing as an effusion.
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7/159. Primary intraocular lymphoma with a low interleukin 10 to interleukin 6 ratio and heterogeneous IgH gene rearrangement.

    Primary intraocular lymphoma is almost always a central nervous system B-cell non-Hodgkin lymphoma. Primary intraocular lymphoma is commonly diagnosed by demonstrating lymphoma cells in the vitreous or cerebrospinal fluid. An interleukin (IL) 10 to IL-6 ratio greater than 1.0 in these fluids and the detection of immunoglobulin gene rearrangement are useful adjuncts in the diagnosis of primary intraocular lymphoma. We report a case of primary intraocular lymphoma diagnosed by chorioretinal biopsy in which no malignant cells were identified in the vitreous and in which the IL-10 to IL-6 ratio was less than 1.0. The detection of IgH gene rearrangement heterogeneity in the tumor cells by polymerase chain reaction, a high tumor mitotic figure rate, and the rapid onset of multiple brain lesions suggest an aggressive malignant neoplasm.
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8/159. T cell malignant lymphoma in the central nervous system after acute lymphoblastic leukemia in a child.

    A malignant lymphoma developed in the central nervous system (CNS) of a 7-year-old boy 5 years after he had received chemotherapy and cranial irradiation for acute lymphoblastic leukemia (ALL). bone marrow analysis of the original leukemia showed a pre-B cell phenotype, whereas the resected brain tumor showed a T cell phenotype on immunophenotypic analysis. Because of this difference in immunophenotype, and because the patient had received multiple-drug chemotherapy and 1,800 cGy of cranial irradiation for the original ALL, we consider that the malignant lymphoma was a second neoplasm. This is a very rare case in two respects: it was a malignant lymphoma arising in the CNS as a second neoplasm after ALL and a T cell lymphoma occurring in a child.
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9/159. Non-Hodgkin's lymphomas involving the uterus: a clinicopathologic analysis of 26 cases.

    Non-Hodgkin's lymphomas (NHL) involving the uterus may be either low-stage neoplasms that probably arise in the uterus (primary) or systemic neoplasms with secondary involvement. In this study, 26 NHL involving the uterus are reported. Ten cases were stage I(E) or II(E) and are presumed to be primary. The mean age of patients at presentation was 55 years (range, 35 to 67 years), and abnormal uterine bleeding was the most frequent complaint (six patients). Nine of 10 tumors involved the cervix. Histologically, eight were diffuse large B-cell lymphoma (DLBCL); one was follicle center lymphoma, follicular, grade 1; and one was marginal zone B-cell lymphoma. At 5 years of clinical follow-up, five of six patients were alive after treatment. In 12 cases, uterine involvement was part of a systemic disease at diagnosis, either stage III(E) or IV. The mean patient age at the time that uterine involvement was detected was 58 years (range, 22 to 75 years); 6 of 12 had abnormal uterine bleeding. Six tumors involved both cervix and corpus, four corpus, and two cervix. Six were DLBCL; two were small lymphocytic lymphoma; three were follicle center lymphoma, follicular, grade 1 (two cases) or grade 2 (one case); and one was precursor T-cell lymphoblastic lymphoma. At 5 years of clinical follow-up, two of seven patients were alive after treatment. Four DLBCL arose in patients with incomplete clinical information; therefore, stage is unknown. We conclude that low-stage (presumably primary) uterine NHL are most commonly DLBCL, predominantly arise in the cervix, and cause abnormal uterine bleeding. High-stage NHL are a heterogeneous group of B-cell neoplasms that can involve the cervix or the corpus.
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10/159. Paraneoplastic pemphigus associated with bronchiolitis obliterans.

    Paraneoplastic pemphigus (PNP) is an autoantibody-mediated mucocutaneous blistering disease associated with underlying neoplasms. autoantibodies of PNP bind to the plakin family of cytoplasmic proteins and desmogleins of cell-surface target antigens. We describe a 36-year-old female patient with PNP who had non-Hodgkin's lymphoma, and who developed bronchiolitis obliterans and died of respiratory failure. autopsy findings confirmed luminal narrowing of bronchioles by scarring, which is a histopathologic features of bronchiolitis obliterans. After the onset of respiratory failure, the reaction of autoantibodies against the plakins detected by immunoprecipitation at the onset of PNP disappeared with negative immunofluorescence within the bronchial epithelium. It is thought that autoantibodies against some of these antigens play a role in causing acute inflammation of the respiratory epithelium. In treating PNP, the possibility of the patient developing the lethal complication bronchiolitis obliterans should be kept in mind. Furthermore, prevention of the initial autoantibody-mediated injury to the respiratory epithelium should be an important treatment goal.
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