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Query: UMLS:C0019693 (HIV)
 170,526 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Plasmablastic lymphoma (PBL) is a rare aggressive variant of large B-cell lymphoma defined as a proliferation of large neoplastic plasmablasts/immunoblasts. PBL was first described as a distinct entity in a group of 16 patients with lymphoma of the oral cavity. Most patients are HIV-positive men. The disease has also been reported in other patient groups, often in association with primary or other acquired immunodeficiency. PBL shows a predilection for the oral cavity, although extraoral involvement also occurs. Because of its rarity, unique clinical features, and overlapping morphologic/immunophenotypic features, care must be taken to distinguish PBL from diffuse large B-cell lymphoma and plasma cell neoplasms with plasmablastic features. We report 3 cases of neoplasms with plasmablastic histomorphology involving the oral cavity. The relevant clinical, morphologic, and immunophenotypic features and treatment are presented, along with a review of the literature.
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PMID:Lymphoid neoplasms of the oral cavity with plasmablastic morphology-a case series and review of the literature. 3149 13

Plasmablastic lymphoma (PBL) is a rare and aggressive malignancy associated with immunosuppression and the oncogenic effects of the Epstein-Barr virus (EBV). We present an HIV-positive man with PBL that presented as ulcers and violaceous exophytic nodules on the legs. The clinical features, histologic appearance, and differential diagnosis of this malignancy are briefly reviewed.
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PMID:Violaceous nodules in an HIV-positive man. 3223 93

HIV-positive patients have a 60- to 200-fold increased incidence of Non-Hodgkin Lymphomas (NHL) because of their impaired cellular immunity. Some NHL are considered Acquired Immunodeficiency Syndrome (AIDS) defining conditions. Diffuse large B-cell Lymphoma (DLBC) and Burkitt Lymphoma (BL) are the most commonly observed, whereas Primary Effusion Lymphoma (PEL), Central Nervous System Lymphomas (PCNSL), Plasmablastic Lymphoma (PBL) and classic Hodgkin Lymphoma (HL) are far less frequent. Multicentric Castleman disease (MCD) is an aggressive lymphoproliferative disorder highly prevalent in HIV-positive patients and strongly associated with HHV-8 virus infection. In the pre-Combination Antiretroviral Therapy (CART) era, patients with HIV-associated lymphoma had poor outcomes with median survival of 5 to 6 months. By improving the immunological status, CART extended the therapeutic options for HIV positive patients with lymphomas, allowing them to tolerate standard chemotherapies regimen with similar outcomes to those of the general population. The combination of CART and chemotherapy/ immuno-chemotherapy treatment has resulted in a remarkable prolongation of survival among HIVinfected patients with lymphomas. In this short communication, we briefly review the problems linked with the treatment of lymphoproliferative diseases in HIV patients. Combination Antiretroviral Therapy (CART) not only reduces HIV replication and restores the immunological status improving immune function of the HIV-related lymphomas patients but allows patients to deal with standard doses of chemotherapies. The association of CART and chemotherapy allowed to obtain better results in terms of overall survival and complete responses. In the setting of HIVassociated lymphomas, many issues remain open and their treatment is complicated by the patient's immunocompromised status and the need to treat HIV concurrently.
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PMID:HIV-Related Lymphoproliferative Diseases in the Era of Combination Antiretroviral Therapy. 3229 49

Plasmablastic lymphoma (PBL) is a CD20-negative large B-cell lymphoma with a plasmacytic phenotype and a dismal prognosis, which has been defined as a distinct entity only in the 2008 WHO Classification of Haematopoietic and Lymphoid Tissue and confirmed in the 2017 Edition. Current knowledge of the biological, clinical and prognostic features of PBL is mostly limited, resulting in diagnostic issues, as well as in lack of standard of care and effective therapeutic options. PBL commonly affects the oral cavity of HIV-positive individuals, however the gastrointestinal (GI) tract is the most common extraoral site, and in this location most patients are HIV-negative. In this review, we focus on the clinical, morphological and prognostic features of PBL arising in the GI tract, in order to improve knowledge on this rare, but aggressive disease.
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PMID:Clinical, pathological and molecular features of plasmablastic lymphoma arising in the gastrointestinal tract: A review and reappraisal. 3286 11

Plasmablastic lymphoma is a rare subtype of non-Hodgkin's lymphoma, which generally presents an aggressive clinical course and low survival rates. It is strongly associated with HIV infection and the most common site of involvement of the territory of the head and neck is Waldeyer's lymphatic ring, followed by the gastrointestinal tract, lymph nodes and skin. The morphological characteristics of PBL in the oral cavity / jaw in the context of HIV infection show diffuse sheets of large immunoblastic cells with abundant cytoplasm, vesicular chromatin and prominent nucleus, a small located in the center with plasma cells differentiation. The main goal of this article is to review the literature of the plasmablastic lymphoma and report a case. Key words:Plasmablastic lymphoma, PBL, HIV, AIDS, Non Hodgkin Lynphoma.
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PMID:Plasmablastic lymphoma as a manifestation of the human immunodeficiency virus: Case report. 3238 94

Plasmablastic lymphoma (PBL) is a rare and aggressive B-cell Non-Hodgkin lymphoma (NHL) associated with immunocompromised states such as HIV. We present a case of PBL in an HIV patient presenting as spontaneous tumor lysis syndrome and discuss the clinical challenges hence encountered.
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PMID:An HIV Associated Plasmablastic Lymphoma With Spontaneous Tumor Lysis Syndrome. 3292 26

Plasmablastic lymphoma is an aggressive variant of lymphomas recently distinct from diffuse large B cell lymphoma. It has been initially described as a disease affecting the oral cavity of immunocompromised patients. We here report the first case of a 54-year-old patient with nasal septum nodule, bleeding on contact and after sneezing which occurred 6 months before admission. Facial computed tomography (CT) scan showed thickening of the nasal mucosa of 14mm. Excisional biopsy showed tumor proliferation composed of plasmablastic cells with immunophenotypic features: CD 138+, ki67 80%, EMA+, CD79a+, CD 56+. Staging and HIV serology were negative. Given the rarity of this lymphoma there are no standard treatments and most patients have treatment-resistant lymphoma with poor prognosis. Our patient received 6 cycles of CHOP-like chemotherapy associated with 40 gy radiotherapy in 20 fractions of 2 gy with complete remission (unusual in the cases described in the literature).
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PMID:[Isolated plasmablastic lymphoma of nasal mucosa in an immunocompetent patient achieving complete remission after multimodal treatment: about an African patient and literature review]. 3306 24

Plasmablastic lymphoma (PBL) is an aggressive B-cell non-Hodgkin lymphoma associated with immunodeficiency in the context of Human Immunodeficiency Virus (HIV) infection or iatrogenic immunosuppression. While a rare disease in general, the incidence is dramatically increased in regions of the world with high HIV prevalence. The molecular pathogenesis of this disease is poorly characterized. Here, we defined the genomic features of PBL in a cohort of 110 patients from South Africa (15 by whole exome sequencing and 95 by deep targeted sequencing). We identified recurrent mutations in genes of the JAK-STAT signaling pathway, including STAT3 (42%), JAK1 (14%) and SOCS1 (10%), leading to its constitutive activation. Moreover, 24% of cases harbored gain-of-function mutations in RAS family members (NRAS and KRAS). Comparative analysis with other B-cell malignancies uncovered PBL-specific somatic mutations and transcriptional programs. We also found recurrent copy number gains encompassing the CD44 gene (37%), which encodes for a cell surface receptor involved in lymphocyte activation and homing, and was found expressed at high levels in all tested cases, independent of genetic alterations. These findings have implications for the understanding of the pathogenesis of this disease and the development of personalized medicine approaches.
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PMID:Genomic characterization of HIV-associated plasmablastic lymphoma identifies pervasive mutations in the JAK-STAT pathway. 3322 11

Plasmablastic lymphoma (PBL) is a rare and clinically aggressive neoplasm that typically occurs in immunocompromised individuals, including those with HIV infection and solid organ allograft recipients. Most prior studies have focused on delineating the clinicopathologic features and genetic attributes of HIV-related PBLs, where MYC deregulation and EBV infection, and more recently, mutations in JAK/STAT, MAP kinase, and NOTCH pathway genes have been implicated in disease pathogenesis. The phenotypic spectrum of post-transplant (PT)-PBLs is not well characterized and data on underlying genetic alterations are limited. Hence, we performed comprehensive histopathologic and immunophenotypic evaluation and targeted sequencing of 18 samples from 11 patients (8 males, 3 females, age range 12-76 years) with PT-PBL; 8 de novo and 3 preceded by other types of PTLDs. PT-PBLs displayed morphologic and immunophenotypic heterogeneity and some features overlapped those of plasmablastic myeloma. Six (55%) cases were EBV+ and 5 (45%) showed MYC rearrangement by fluorescence in situ hybridization. Recurrent mutations in epigenetic regulators (KMT2/MLL family, TET2) and DNA damage repair and response (TP53, mismatch repair genes, FANCA, ATRX), MAP kinase (KRAS, NRAS, HRAS, BRAF), JAK/STAT (STAT3, STAT6, SOCS1), NOTCH (NOTCH1, NOTCH3, SPEN), and immune surveillance (FAS, CD58) pathway genes were observed, with EBV+ and EBV- cases exhibiting similarities and differences in their mutational profiles. Clinical outcomes also varied, with survival ranging from 0-15.9 years postdiagnosis. Besides uncovering the biological heterogeneity of PT-PBL, our study highlights similarities and distinctions between PT-PBLs and PBLs occurring in other settings and reveals potentially targetable oncogenic pathways in disease subsets.
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PMID:Phenogenomic heterogeneity of post-transplant plasmablastic lymphomas. 3329 69


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