Cases reported "Lymphoma, AIDS-Related"

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1/40. Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient.

    Intravascular lymphomatosis (IL) is an unusual neoplasm characterized by multifocal proliferation of lymphoma cells exclusively within the blood vessels. We report here a patient with acquired immunodeficiency syndrome (AIDS) and disseminated Kaposi's sarcoma. A 233-bp amplification product of HHV-8 was detected in the dna extracted from specimens of Kaposi's sarcoma at different sites by polymerase chain reaction (PCR). At autopsy, the vessels within the Kaposi's sarcoma were dilated and filled with atypical large mononuclear cells. No such feature was seen in the vessels of non-Kaposi's sarcomatous regions. Immunohistochemically, the spindle cells of Kaposi's sarcoma were positive for CD31 (endothelial cell marker). The intravascular tumor cells were positive for CD45 (leukocyte common antigen) but negative for others, including chloroacetate esterase, CD45-RO (UCHL-1, Pan-T), CD3, CD43, CD20 (L26, Pan-B), CD30 (Ki-1), immunoglobulin heavy chains and light chains, CD56 (natural killer cell antigen), and CD31. Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in the dna extracts from fresh tissue of Kaposi's sarcoma by PCR, which indicated that the lymphoma cells within the Kaposi's sarcoma were of monoclonal B cell origin. in situ hybridization revealed that EBER-1 transcripts were present in the lymphoma cells of IL but not in the spindle cells of Kaposi's sarcoma. To the authors' best knowledge, this is the first instance of IL in an AIDS patient with direct evidence of EBV association.
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2/40. 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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3/40. 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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4/40. True histiocytic lymphoma of the esophagus in an HIV-positive patient: an ultrastructural study.

    A 56-year-old white woman, seropositive for human immunodeficiency virus for 18 months without signs of acquired immunodeficiency syndrome, presented with retrosternal pain and progressive dysphagia secondary to an exophytic esophageal mass. Biopsies of the tumor showed a malignant neoplasm composed of pleomorphic, noncohesive cells growing in a diffuse, sheet-like fashion. Immunohistochemically, tumor cells were nonreactive with epithelial, lymphoid, neural, and monocyte/macrophage markers. Despite the noncontributory immunohistochemical findings, ultrastructural study of the tumor cells revealed convincing histiocytic features. Individual cells possessed long, slender filopodial projections, prominent golgi apparatus, residual bodies, rare lysosomes, and prelysosomes. Immunoglobulin heavy chain and T-cell receptor gamma gene rearrangement studies detected no evidence of a clonal gene rearrangement. The patient responded poorly to chemotherapy and died 5 months after her initial symptom of dysphagia.
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5/40. Localized oral fusarium infection in an AIDS patient with malignant lymphoma.

    We report here a case of localized oral fusarium infection in an AIDS patient who developed an ulceration in the soft palate. fusarium solani was identified by histopathology and culture. We believe this to be the second reported case of oral fusarium infection in a patient with haematological malignancy and the first reported association of oral fusarium infection with AIDS.
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6/40. Human herpesvirus 8-associated solid lymphomas that occur in AIDS patients take anaplastic large cell morphology.

    Human herpesvirus type 8 (HHV-8; Kaposi's sarcoma-associated herpesvirus) is a recently isolated human herpesvirus frequently identified in Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. Here we report three cases of HHV-8-bearing solid lymphomas that occurred in AIDS patients (Cases 1-3). All three patients were homosexual men presenting extranodal masses in the lungs (Case 1) or skin (Cases 2 and 3), together with the presence of Kaposi's sarcoma (Case 1), primary effusion lymphoma (Case 2), or multicentric Castleman's disease (Case 3). These solid lymphomas exhibited anaplastic large cell morphology and expressed CD30, corresponding to the recent diagnostic criteria of anaplastic large cell lymphoma (ALCL). The chromosomal translocation t(2;5)-associated chimeric protein p80NPM/ALK was not observed in any of these cases. HHV-8 was detected in all of these cases by polymerase chain reaction, immunohistochemistry of HHV-8-encoded ORF73 protein, and in situ hybridization of T1.1. Epstein-Barr virus was detected only in Cases 2 and 3 by in situ hybridization. It is interesting that inoculation of a cell line obtained from a primary effusion lymphoma cell in Case 2 to severe combined immunodeficiency mice produced HHV-8-positive and Epstein-Barr virus-negative tumors in inoculated sites. These tumor cells exhibited phenotypes of ALCL that were identical to the subcutaneous tumor cells of this particular patient. These findings clearly show that HHV-8 can associate with solid lymphomas and that it can take anaplastic large cell morphology. Those lymphomas should be distinguished from the classical ALCL as were defined by the revised European-American classification of lymphoid neoplasms even though morphology and a part of immunophenotype mimic that of classical ALCL.
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7/40. Isolated primary hepatic lymphoma in a patient with acquired immunodeficiency syndrome.

    Non-Hodgkin lymphoma (NHL) of the B-cell type is the second most common neoplasm in patients with human immunodeficiency virus (HIV) infection after Kaposi sarcoma (KS). The majority of cases of NHL in patients with acquired immunodeficiency syndrome (AIDS) involve extranodal sites; most frequently the gastrointestinal tract (GIT) and the central nervous system (CNS). Hepatic NHL in patients with AIDS was first described by Reichert et al in 1983 in an autopsy series. It usually presents with multiple large hepatic masses and involvement of other abdominal organs or lymph nodes. The authors present a case of primary hepatic NHL in a patient with AIDS, presenting with innumerable small intrahepatic masses without the involvement of any other organs.
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8/40. HIV-associated malignancies in children.

    The exact incidence of malignancies in HIV-infected children is not clear; however, an excess of non-Hodgkin's lymphomas (NHLs) and soft tissue tumors (leiomyosarcomas) is evident. The spectrum of diseases is slightly different in children compared to adults. For example, Kaposi's sarcoma (KS), although common in HIV-infected adults, is rare in children in industrialized countries. Preliminary results with brief, dose-intensive chemotherapeutic regimens have been encouraging. Such regimens may be complicated, however, by multiple HIV-associated organ dysfunctions, drug interactions, and infectious complications secondary to severe immunosuppression.
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9/40. Clinicopathological characterization of an hiv-2-infected individual with two clonally unrelated primary lymphomas.

    Human immunodeficiency virus 2 (hiv-2) is endemic in West africa and is a causative agent of the acquired immunodeficiency syndrome. Only a small number of hiv-2-infected patients have been described in detail. Non-Hodgkin's lymphoma (NHL) is the second most common neoplasm occurring in hiv-1-infected patients, but its incidence seems to be lower in hiv-2-infected individuals. We report an hiv-2-infected patient from cape verde (West africa) with separate and distinct systemic and primary central nervous system large B-cell lymphomas and review the findings of cases of hiv-2-associated lymphomas reported in the literature. Different clonal rearrangements of the immunoglobulin heavy chain gene could be detected in the two lymphomas of our patient by polymerase chain reaction and sequence analysis. These data indicate the presence of two clonally unrelated large B-cell lymphomas in the same patient, which is an unusual finding. Neither Epstein-Barr virus nor human herpesvirus 8 could be detected in the tumor tissues or the cerebrospinal fluid. hiv-2 infection should be considered in patients with NHL, especially in those from West africa.
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10/40. Primary hepatic anaplastic large-cell lymphoma of T-cell phenotype in acquired immunodeficiency syndrome: a report of an autopsy case and review of the literature.

    Anaplastic large-cell lymphomas (ALCL) were first described by Stein et al. in 1985 as large-cell neoplasms with a pleomorphic appearance, subtotal effacement of the lymph node structure, and expression of the lymphoid activation antigen CD-30 (Ki-l). Since their first description, these tumors have been documented in a variety of extranodal sites. We report a primary hepatic anaplastic large-cell lymphoma in a patient with advanced AIDS, who presented with hepatic failure and multiple nodules in the liver. A complete autopsy showed discrete tumor nodules throughout the entire liver without gross or microscopic involvement of lymph nodes or any other organs by the neoplastic process. The tumor cells showed typical histological and immunohistochemical features of ALCL and were strongly immunoreactive with the T-cell markers CD-3 and UCHL-1. Only one previous case of primary hepatic ALCL has been reported in the literature, and this tumor occurred in an immunocompetent patient and was not immunoreactive for B- or T-cell markers. To our knowledge, this study represents the first reported case of primary hepatic anaplastic large-cell lymphoma of T-cell phenotype. Additionally, this is the first case of primary hepatic ALCL reported in an AIDS patient.
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