Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Immunoblastic lymphoma developed in a patient diagnosed as having angioimmunoblastic lymphadenopathy 5 years earlier. Chemotherapy schedules including alkylating agents were administered for 1 year, resulting in subsequent remission of the lymphoma. Thirty months after the start of chemotherapy, preleukemic changes were observed in peripheral blood and bone marrow, and acute nonlymphocytic leukaemia (M5 of the FAB classification) was diagnosed shortly afterwards. The possible role of both chemotherapy and the immunodeficiency associated to angioimmunoblastic lymphadenopathy in the pathogenesis of the leukemia is discussed.
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PMID:Sequential appearance of immunoblastic lymphoma and acute nonlymphocytic leukemia in a patient with angioimmunoblastic lymphadenopathy. 393 63

Kaposi's sarcoma (KS), common among persons with acquired immunodeficiency syndrome (AIDS), is caused by KS herpesvirus (KSHV) but whether KSHV causes other malignancies is uncertain. Using linked United States AIDS and cancer registries, we measured the incidence of specific malignancies in persons with AIDS (4-27 months after AIDS onset). We identified associations with KSHV by calculating a relative risk: cancer incidence in persons with KS (all were KSHV-infected) divided by incidence in persons without KS. Using Poisson regression, relative risks were adjusted for human immunodeficiency virus risk group, gender, age, race, and calendar year. We included 189 159 subjects (26 972 with KS). Immunoblastic lymphoma was significantly associated with KS (506 cases; relative risks: unadjusted 2.44, 95%CI 2.00-2.96, adjusted 1.58, 95%CI 1.29-1.93). Only one immunoblastic lymphoma had pleura as primary site. None of 37 other specified malignancies (other non-Hodgkin lymphomas, haematological malignancies, solid tumours) was significantly associated with KS. In summary, the association of immunoblastic lymphoma with KS was specific among examined malignancies and remained significant after statistical adjustment. Our findings, and the previously demonstrated presence of KSHV in the histologically related primary effusion lymphoma, suggest that KSHV is involved in the pathogenesis of some immunoblastic lymphomas.
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PMID:Cancers associated with Kaposi's sarcoma (KS) in AIDS: a link between KS herpesvirus and immunoblastic lymphoma. 1172 Apr 64

Plasmablastic lymphoma (PBL), an aggressive non-Hodgkin's lymphoma that carries a poor prognosis, previously has been identified almost exclusively in patients infected with the human immunodeficiency virus (HIV). We present a case of a 42-year-old HIV-negative patient presenting with an isolated nasal cavity mass, the typical presentation for PBL. The patient was given systemic chemotherapy, central nervous system prophylaxis, and consolidative locoregional radiotherapy and achieved a complete clinical response. This case suggests PBL should be considered in HIV-negative patients with characteristic findings.
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PMID:Plasmablastic lymphoma presenting in a human immunodeficiency virus-negative patient: a case report. 1278 13

Infection with human immunodeficiency virus (HIV) increases the risk of developing non-Hodgkin lymphoma. Plasmablastic lymphoma (PBL) is a rare variant of diffuse large cell lymphoma that often involves the oral cavity of HIV+ patients. It is characterized by immunoblastic morphology and plasma cell phenotype. Cutaneous involvement in PBL appears to be rare. We report a 44-year-old man with AIDS and Kaposi sarcoma (KS) previously treated with doxorubicin who, following treatment with highly active antiretroviral therapy, developed an erythematous infiltrated nodule on the right arm. Histology showed subcutaneous fat necrosis and clusters of atypical large plasma cells (plasmablastic cells). Immunohistochemistry revealed lambda light chain restriction. Epstein-Barr virus (EBV) mRNA was detected by in situ hybridization within the plasmablastic cells. Polymerase chain reaction amplification with specific primers for human herpesvirus 8 (HHV-8) performed on the skin biopsy specimen detected a specific band. A complete screening (bone marrow biopsy, computed tomographic scan, radiological survey) disclosed no abnormalities. The lesion resolved spontaneously after 3 months. Two years later an infiltrated plaque developed on the abdominal wall. The clinical and histopathological features of this new lesion were similar to those observed 2 years previously. No evidence of extracutaneous involvement was detected. The lesion again resolved spontaneously after 25 days. PBL may be seen in patients with transplants or receiving chemotherapy, but is usually observed in patients with advanced AIDS. The observation of recurrent self-healing EBV- and HHV-8-associated cutaneous monoclonal plasmablastic infiltrates, in a patient with AIDS and KS, expands the clinical spectrum of AIDS-associated plasmablastic lymphoproliferative disorders.
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PMID:Recurrent and self-healing cutaneous monoclonal plasmablastic infiltrates in a patient with AIDS and Kaposi sarcoma. 1618 70

Plasmablastic lymphoma (PBL) is an uncommon, recently described B-cell-derived lymphoma that displays distinctive affinity for extranodal presentation in the oral cavity. Plasmablastic lymphoma is strongly associated with human immunodeficiency virus (HIV) infection, but has been reported in HIV-negative individuals. Plasmablastic lymphoma may be poorly recognized by pathologists, which is partly attributable to its relatively rare occurrence and unusual immunophenotype. Five cases of oral cavity lymphomas conforming to the current World Health Organization morphological criteria for PBL were retrieved from the consultation files at the Armed Forces Institute of Pathology. An immunohistochemical panel consisting of CD3, CD20, CD30, CD38, CD45RB, CD79a, CD138, Bcl-2, Bcl-6, Alk-1, Ki-67, EBV-LMP-1, and HHV8 was performed. All 5 cases were immunoreactive for CD38 and/or CD138, confirming plasma cell differentiation of the tumor cells. CD20 was immunoreactive in 1 case, and CD79a was positive in 2 cases. HHV8 and EBV-LMP-1 were nonreactive in all cases. Follow-up revealed only 1 patient alive with no evidence of disease. Our cases show that PBL is an aggressive type of B-cell lymphoma predominantly found in the oral cavity. Plasmablastic lymphoma is often associated with HIV infection.
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PMID:Plasmablastic lymphoma: a clinicopathologic correlation. 1641 38

Plasmablastic lymphoma (PBL) is an unusual subtype of human immunodeficiency virus (HIV)-related diffuse large B-cell lymphoma that was first described in the oral cavity. HIV-related lymphomas are frequently associated with Epstein-Barr virus (EBV). Recently, dual infection with EBV and human herpesvirus 8 (HHV8) has been demonstrated in PBL. So far, a few cases of PBL occurring in an HIV-negative patient have been documented and all of them were associated with immunosuppression status and/or EBV infection. Here we report a EBV and HHV8-negative oral PBL occurring in an immunocompetent HIV-negative male, which would be the first case.
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PMID:Epstein-Barr virus and human immunodeficiency virus-negative oral plasmablastic lymphoma. 1676 21

Plasmablastic lymphoma is an aggressive diffuse large B-cell lymphoma classically arising in the oral cavities and jaws of individuals infected with the human immunodeficiency virus (HIV). More recently, cases of plasmablastic lymphoma have been identified in individuals negative for HIV. We report a case of plasmablastic lymphoma presenting as a rapidly expanding upper extremity mass in a 66-year-old individual negative for HIV. Aggressive multiple-agent chemotherapy resulted in a dramatic improvement of all symptoms. Increasing awareness of plasmablastic lymphoma in individuals who are HIV negative can allow for a better understanding of its clinical course and for specific chemotherapeutic regimens to be developed.
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PMID:Plasmablastic lymphoma presenting as an arm mass in an individual negative for human immunodeficiency virus: a case report. 1679 82

Plasmablastic lymphoma is an aggressive subtype of diffuse large B-cell lymphoma that is mainly observed in patients with the human immunodeficiency virus (HIV) infection, and it tends to arise in the oral cavity. We present a case of an HIV-infected patient with plasmablastic lymphoma with prolonged survival. The 30-yr-old woman was found to have an oral lesion at the time of the diagnosis of HIV infection. Histological and immunochemical examination of biopsy of the oral lesion showed plasmablastic lymphoma (CD138+). She received two cycles of cyclophosphamide, vincristine, doxorubicin, and prednisolone (CHOP) that started 10 weeks after the initiation of antiretroviral therapy. The continuing pancytopenia and an adenoviral febrile infection did not permit further antineoplastic treatment. A gradual decrease of the oral lesion was noted after the second cycle of chemotherapy that led to the disappearance of the lesion 7 months later. The patient remains in complete remission 61 months after the diagnosis of plasmablastic lymphoma.
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PMID:Prolonged survival of an HIV-infected patient with plasmablastic lymphoma of the oral cavity. 1709 93

Plasmablastic lymphoma was originally described in 1997 by Delecluse et al. and is an aggressive variant of diffuse large B-cell non-Hodgkin lymphoma seen predominantly in a setting of acquired immunodeficiency syndrome and nearly always in extranodal sites. The authors have seen 10 cases in their department between 2001 and 2005. The patients' ages ranged from 24 to 39 years and there were 7 females and 3 males. In 7 cases where human immunodeficiency virus had been tested it was positive. Eight cases were extranodal and 2 cases involved lymph nodes. Five cases were followed up and 4 were confirmed dead within 5 months of diagnosis, verifying the aggressive nature of this condition. Histology showed large, polygonal tumor cells some of which had a slightly plasmacytic appearance. Six cases had a "starry sky" background. Immunohistochemical stains were negative in 1 case, while the other 9 cases were positive for CD138. Electron microscopy showed concentrically arranged rough endoplasmic reticulum in the cytoplasm in 9 cases. In 1 case the cells were too degenerate for evaluation. This study shows that the ultrastructural features are well developed and can help in distinguishing plasmablastic lymphoma from other light microscopically undifferentiated tumors.
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PMID:The ultrastructural features of plasmablastic lymphoma. 1718 35

Plasmablastic lymphoma is a rare subcategory of non-Hodgkin lymphoma frequently associated with human immunodeficiency virus. It is a large B-cell lymphoma that has a predilection for the oral cavity. Clinically, plasmablastic lymphoma may mislead to a diagnosis of Kaposi's sarcoma. When infected, plasmablastic lymphoma may mimic an odontogenic cellulitis. Epstein-Barr virus and human herpesvirus 8 are very often associated. Awareness of this entity can prevent misdiagnosis with nonlymphoid malignancies, notably Kaposi's sarcoma, because this lesion does not express the conventional B-cell markers. Unfortunately, as for other high-grade lymphomas in patients with acquired immunodeficiency syndrome (AIDS), the prognosis is poor. The case of a heterosexual 42-year-old man referred for a right hemifacial neoplasm is reported.
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PMID:Plasmablastic lymphoma: a case report. 1726 56


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