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)

Plasmablastic lymphoma of the oral cavity is a form of non-Hodgkin lymphoma (NHL) and was first described in 1997. We describe a case of plasmablastic lymphoma in an HIV-infected patient who presented with an expanding oral lesion and symptoms of a toothache. We review all cases of plasmablastic lymphoma that have been reported in the literature. Plasmablastic lymphoma is strongly associated with immunodeficiency, and most particularly, with HIV infection. The pathophysiological origin of plasmablastic lymphoma has not been fully characterised, but the presence of Epstein-Barr virus (EBV) has often been documented in biopsy specimens, supporting a role for EBV in the pathogenesis of this lymphoma. The differential diagnosis for an expanding oral lesion includes both infectious and malignant processes. Biopsy is essential for making a correct and prompt diagnosis. Treatment usually involves chemotherapy, but antiretroviral therapy may also have an important role. Infectious disease clinicians should be aware of this newly described and increasingly encountered lymphoma, since it is prominently associated with immunosuppression and may be mistaken for other entities.
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PMID:Plasmablastic lymphoma of the oral cavity: a rapidly progressive lymphoma associated with HIV infection. 1835 67

Plasmablastic lymphoma (PBL) is a rare form of diffuse large B-cell lymphoma characterized by weak/absent expression of conventional B-cell markers and strong expression of plasma cell markers. It is strongly associated with human immunodeficiency virus (HIV) and Epstein Barr virus infection, and shows an unusual tropism to the oral cavity. Herein we describe a patient with AIDS who presented with weight loss and dysphagia owing to a large gastroesophageal mass. His radiographic and endoscopic findings and long history of cigarette consumption suggested carcinoma. Biopsy demonstrated a poorly differentiated tumor stained negatively to routine lymphoid markers including CD20. However, gene rearrangement studies confirmed a B-cell process and a more detailed immunohistochemical analysis revealed the cells stained positively for CD138 (plasma cell antigen). These findings were diagnostic of PBL. Our report reviews the wide differential diagnosis of PBL and underscores the importance of a broad array of viral and molecular studies needed to establish this diagnosis.
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PMID:AIDS-associated plasmablastic lymphoma presenting as a poorly differentiated esophageal tumor: a diagnostic dilemma. 1866 32

Plasmablastic lymphoma (PBL) is a distinct subtype of non-Hodgkin B-cell lymphoma, originally described with a strong predilection to the oral cavity of human immunodeficiency virus (HIV)-infected individuals. Data regarding patient age and gender, HIV status, initiation of and response to highly active antiretroviral therapy (HAART), tumor extent, pathology, treatment, and outcome were extracted from 112 cases of PBL identified in the literature. The median age at presentation was 38 years with a male predominance of 7:1, and the median CD4+ count was 178 cells/mm(3). PBL presented on average 5 years after diagnosis of HIV. Common primary sites of presentation included the oral cavity, gastrointestinal tract, and lymph nodes. Most cases presented with either stage I or stage IV disease. There was a variable expression of B-cell markers in tumor cells, but plasma cell markers were expressed in all cases. EBV was detected in 74%. Chemotherapy was used to treat 55% patients and was combined with radiotherapy in 21% cases. Complete response was obtained in 66% of treated cases; the majority of these responses were seen after CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). The refractory/relapsed disease rate was 54%. Death occurred in 53% of patients, with a median overall survival of 15 months. Sex, CD4+ count, viral load, clinical stage, EBV status, primary site of involvement, and use of CHOP failed to show an association with survival. PBL is an aggressive B-cell lymphoma that presents in both oral and extra-oral sites of chronically HIV-infected immunosuppressed young men.
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PMID:HIV-associated plasmablastic lymphoma: lessons learned from 112 published cases. 1875 46

Plasmablastic lymphoma (PBL) is very rare, and predominantly occurs in Human immunodeficiency virus (HIV)-positive individuals. It shows a strong affinity for the oral cavity and for the Epstein-Barr virus (EBV) positive. We investigated the clinicopathologic characteristics of six cases of PBL in Koreans. All patients were HIV-negative and without underlying immunodeficiency. The age distribution was bimodal, and four patients were older than 60 years. Male predominance was observed with male to female ratio of 5:1. The organs primarily involved were the terminal ileum, stomach, oral cavity, tonsil, nasal cavity and meninges. The tumors were histologically typical of PBL. Three of them were composed of monomorphic large immunoblastic or plasmablastic cells, and classified as PBL of the oral mucosa type. Another three cases were classified as PBL with plasmacytic differentiation. Five cases revealed loss of B-cell antigens with CD138 or MUM1 substitution. CD10 was positive in two cases (PBLs of the oral mucosa type), and one of them unexpectedly expressed cytokeratin. EBV was detected in one case (PBL with plasmacytic differentiation). Four patients succumbed to PBL in a relatively short period of time. We suggest that PBL is not strongly associated HIV or EBV in Koreans, and that it shows a variable organ distribution without an oro-nasal predilection.
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PMID:Human immunodeficiency virus-negative plasmablastic lymphoma in Korea. 1937 56

Plasmablastic lymphoma (PBL) of the oral cavity is an uncommon, recently described B-cell derived lymphoma that is most commonly seen in patients with human immunodeficiency virus (HIV) infections. The authors report a rare case of PBL in the oral cavity of a 40-year-old man with HIV. The lymphoma cells were positive for leukocyte common antigen, CD79a, CD138, Epstein-Barr virus (EBV) and kappa light chain restriction and negative for CD20, CD3, S100, HMB45 and cytokeratins. The lesion regressed after treatment with local radiotherapy and systemic chemotherapy. The features of this rare disease are summarized based on a comprehensive review of the epidemiological, clinical and immunohistochemical findings of previously reported cases.
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PMID:Plasmablastic lymphoma of the oral cavity in an HIV-positive patient: a case report and review of literature. 1944 81

Plasmablastic lymphoma (PBL) is a recently identified entity that is considered to be a type of diffuse large B-cell lymphoma with a unique immunophenotype and a predilection for the oral cavity of patients with the human immunodeficiency virus (HIV). Although its clinical features may help in the differential diagnosis, an extraoral location in a patient without HIV makes it more difficult to suspect clinically. This case report is the first to describe a patient with PBL originating from the jejunum in a 60-yr-old, HIV-seronegative man. Computed tomography of the face, chest and abdomen showed about a 9.4 x 9.0 cm mass of the proximal jejunum, multiple masses in the musculoskeletal soft tissue, and multiple lymphadenopathies. The histological examinations demonstrated a large cell lymphoma with plasmablastic differentiation. The neoplastic cells were diffusely positive for MUM1, epithelial membrane antigen and lambda light chains, and focally positive for CD79a; but negative for CD3, CD20, CD30, CD34, CD45RO, CD56, CD99, and CD117. The proliferation index by Ki-67 immunohistochemistry was approximately 70%. These findings were compatible with the diagnosis of PBL. The findings in this case suggest that PBL should be included in the differential diagnosis of a small bowel mass even in a HIV-negative patient.
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PMID:A case report with plasmablastic lymphoma of the jejunum. 2019 Oct 56

Plasmablastic lymphoma, which is considered a subtype of diffuse large B-cell lymphoma, shares many similar morphological and immunophenotypic features with plasmablastic transformation of plasma cell myeloma. In the setting of human immunodeficiency virus (HIV) infection, both types of neoplasms can be associated with Epstein-Barr virus (EBV), thus making their distinction challenging. Moreover, the biological relationship between these entities remains unclear. We report four unique cases of plasmablastic lymphoma occurring in the setting of HIV infection that had overlapping clinical and genetic features with plasma cell myeloma. We reviewed the clinical, morphological, and cytogenetic findings and performed immunohistochemistry, in situ hybridization for EBV, chromosome analysis, and fluorescent in situ hybridization (FISH) using the MYC break-apart rearrangement probe. All patients were males with a median age of 45 years. In addition to extra-nodal disease, plasmablastic morphology, and phenotype typical of plasmablastic lymphoma, three of the four cases also showed clinical findings overlapping with plasma cell myeloma, that is, monoclonal serum immunoglobulin and lytic bone lesions. Furthermore, these cases showed complex cytogenetic changes that are more commonly observed in plasma cell myeloma. A unique feature was the presence of MYC (8q24.1) rearrangement confirmed by FISH in all four cases. MYC translocation has been associated with tumor progression in multiple myeloma but has only rarely been previously reported in plasmablastic lymphoma. These cases show a clinical and biological relationship between plasmablastic lymphoma and the plasmablastic variant of plasma cell myeloma. Dysregulation of MYC may be a common genetic mechanism that imparts plasmablastic morphology and aggressive clinical course to B-cell neoplasms at a later stage of differentiation.
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PMID:Plasmablastic lymphoma with MYC translocation: evidence for a common pathway in the generation of plasmablastic features. 2034 82

Plasmablastic lymphoma (PBL) is a diffuse large B-cell lymphoma, a new entity, the incidence of which is increasing, predominantly in the oral cavity of immunocompromised patients, most of whom are infected with human immunodeficiency virus (HIV); only four cases have been reported in non-HIV-infected patients. This report details two cases of PBL of the oral cavity in immunocompetent patients. In both cases, the tumour manifested as a solitary, firm mandibular swelling with dense infiltration of plasma and blastoid cells in the stroma with strong positivity for CD 138 and negativity for CD 20. The patients received radiotherapy and chemotherapy, which resulted in complete remission of the lesion. This is the only case report of two cases of PBL in the oral cavity in immunocompetent patients in which both patients received treatment and showed complete remission of the lesion.
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PMID:Plasmablastic lymphoma of the oral cavity in immunocompetent patients: report of two cases. 2053 63

Plasmablastic lymphoma (PBL) is a rare lymphoma that is characterized by a diffuse proliferation of large neoplastic cells resembling B immunoblasts, but shows the immunophenotype of plasma cells. PBL is most commonly seen in the oral cavity of human immunodeficiency virus (HIV)-positive patients. Epstein-Barr virus (EBV) may be closely related the pathogenesis of PBL. We report a case of HIV-negative PBL in a 75-year-old man without EBV infection. Histologic examination of the cecal tumor following right hemicolectomy and cytologic examination of ascitic fluid were performed. Cytologic specimens were hypercellular and composed of single cells and loosely formed clusters. Large tumor cells showed plasmacytoid features with basophilic cytoplasm, large nuclei, prominent nucleoli, and focal perinuclear halos. Abnormal mitotic figures were easily identified. On immunohistologic study, the tumor cells were positive for CD138 (plasma cell marker) and kappa, but negative for CD45, CD3, CD20, CD79a, CD56, and cyclin D1. The proliferation index (Ki-67) was high. This is a very rare case of PBL without HIV and EBV infection, involving the cecum.
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PMID:Plasmablastic lymphoma of the cecum: report of a case with cytologic findings. 2060 80

Plasmablastic lymphoma (PBL), initially characterized as an aggressive lymphoma arising in the jaw and oral mucosa in HIV-infected patients, was recently reported to occur with extraoral manifestations, heterogeneous histologic findings, and variable association with immunodeficiency states. We reviewed clinical, morphologic, and immunophenotypic features of 13 cases of PBL to determine whether these different subtypes represent distinct morphologic and clinical entities. Two distinct subtypes of PBL were identified and classified as oral and extraoral PBL. The oral PBLs were strongly associated with HIV infection and commonly demonstrated plasmablastic morphologic features without plasmacytic differentiation. Extraoral PBLs tended to occur in patients with underlying non-HIV-related immunosuppression and universally demonstrated plasmacytic differentiation. The patients with oral PBL demonstrated better overall survival compared with patients with extraoral PBL (P = .02). Our findings suggest that PBL with oral and extraoral manifestation represent 2 distinct clinicopathologic entities.
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PMID:Oral and extraoral plasmablastic lymphoma: similarities and differences in clinicopathologic characteristics. 2157 68


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