Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: 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 is a rare and aggressive lymphoma reported to be commonly associated with immunodeficiency state. It possesses a challenge to the clinician owing it aggressiveness and poor prognosis. No standard of care treatment is available for the disease. Here we report a case of an immunocompetant 67 years female who is unique in her presentation as she did not have any of the conventional clinical features and had a history of urothelial carcinoma three months back.
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PMID:Plasmablastic Lymphoma. 2874 29

Plasmablastic lymphoma (PBL) is an aggressive form of non-Hodgkin's lymphoma (NHL) classically seen in patients infected with the human immunodeficiency virus, but can also be seen in other immunocompromised states such as transplant recipients, autoimmune diseases and the elderly. PBL is generally associated with a poor prognosis despite chemotherapy. There is evidence supporting the use of bortezomib in combination with standard chemotherapy to achieve durable responses in patients with PBL. We describe a patient with acquired immunodeficiency syndrome who presented with rectal pain and bright red blood per rectum. He was diagnosed with stage IVA PBL with anorectal, nodal, calvarial and hepatic involvement. Along with highly active antiretroviral therapy, he was treated with six cycles of dose adjusted etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin (DA-EPOCH) plus bortezomib resulting in durable complete remission 30 months after diagnosis.
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PMID:Durable complete remission with combination chemotherapy and bortezomib in HIV-associated plasmablastic lymphoma. 2899 64

Clinical Practice Points. Plasmablastic lymphoma (PBL) is a rare and highly aggressive variant of diffuse large B cell lymphoma with median survival of advanced stage patients varying between 6 and 15 months in previous reports. We report here a human immunodeficiency virus-infected patient surviving over 12 years following treatment for advanced PBL with EPOCH chemotherapy and intrathecal therapy. This case highlights the potential for improved survival in PBL with intensive chemotherapy. Further, literature review suggests promising prospects utilizing novel targeted therapies to increase the rate of prolonged responses.
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PMID:Plasmablastic Lymphoma: Case Report of Prolonged Survival of an Advanced Human Immunodeficiency Patient and Literature Review. 2909 Jan 1

Plasmablastic lymphoma (PBL) is an aggressive lymphoma, often arising in the context of immunodeficiency and associated with Epstein-Barr virus (EBV) infection. The most frequently detected genetic alteration is the deregulation of MYC gene through the translocation - t(8;14)(q24;q32). The diagnosis of PBL is often challenging because it has an overlap in morphology, immunophenotype, cytogenetics and virus association with other lymphomas and plasma cell neoplasms; further, its molecular basis remains elusive. In the present study we aimed to better define the possible contribution of EBV infection as well as miRNA deregulation in PBL pathogenesis. We studied 23 cases of PBL, 19 Burkitt lymphomas (BL), and 17 extra-medullary plasmacytoma (EMPC). We used qPCR and immunohistochemistry to assess EBV latency patterns, while micro-RNA (miRNA) profiling was performed by next generation sequencing (Illumina) and validated by qPCR. Our analysis revealed a non-canonical EBV latency program with the partial expression of some proteins characterizing latency II and the activation of an abortive lytic cycle. Moreover, we identified miRNA signatures discriminating PBL from BL and EMPC. Interestingly, based on the miRNA profile, PBL appeared constituted by two discrete subgroups more similar to either BL or EMPC, respectively. This pattern was confirmed in an independent set of cases studied by qPCR and corresponded to different clinico-pathological features in the two groups, including HIV infection, MYC rearrangement and disease localization. In conclusion, we uncovered for the first time 1) an atypical EBV latency program in PBL; 2) a miRNA signature distinguishing PBL from the closest malignant counterparts; 3) the molecular basis of PBL heterogeneity.
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PMID:MicroRNAs sequencing unveils distinct molecular subgroups of plasmablastic lymphoma. 2929 71

Plasmablastic lymphoma (PBL) is a rare and aggressive subtype of B-cell lymphoma, which occurs typically in the oral cavity of human immunodeficiency virus (HIV)-positive patients. We report a case of a 44-year-old HIV-positive patient with a solitary polypoid mass of the left ureteropelvic junction, causing unilateral hydronephrosis and clinically mimicking urothelial carcinoma. A laparoscopic nephroureterectomy was performed, and pathological examinations revealed the mass as PBL. PBL can present in various forms, even as a polypoid mass of the upper urinary tract, and it should be considered in the differential diagnosis of any mass detected in the HIV-positive patients.
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PMID:Plasmablastic lymphoma presenting as a ureteral polypoid mass. 2969 11

Plasmablastic lymphoma is highly malignant and invasive. It is most commonly found in gastrointestinal tract and is strongly asso- ciated with human immunodeficiency virus (HIV). The authors report a case of a 37-year-old HIV-positive African American woman with a vulvar mass diagnosed as plasmablastic lymphoma. Involvement of the upper gastrointestinal tract had been detected by endoscopic biopsy, suggesting that any patient diagnosed with plasmablastic lymphoma in unusual locations should be investigated for primary lesions in the gastrointestinal tract.
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PMID:Vulvar and gastric involvement in plasmablastic lymphoma. 2995 2

Plasmablastic lymphoma is a rare and aggressive variant of diffuse large B-cell lymphoma with plasmablastic features, which commonly occurs in the oral cavity of human immunodeficiency virus-positive patients. Here, we present a case of plasmablastic lymphoma involving the uterus in a 54-year-old human immunodeficiency virus-negative female patient. The torso positron emission tomography/CT scan revealed intense ¹⁸F-fludeoxyglucose uptake in a bulky uterus with multiple sites of metastatic disease including peritoneal seeding.
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PMID:Human immunodeficiency virus (HIV)-negative uterine plasmablastic lymphoma on ¹⁸F-FDG PET/CT. 3036 72

Plasmablastic lymphoma (PBL) is a distinctly rare neoplasm believed to arise from post-germinal center, terminally differentiated, activated B cells before transformation to plasma cells; and predominantly affecting human immunodeficiency virus (HIV) infected or immunodeficient males. Here, we report a rare case of primary PBL of bone marrow in an immunocompetent male, the diagnosis of which is complicated by the overlapping morphology and immunophenotype with several large cell lymphomas and plasma cell neoplasms; and showing dramatic response to anti-CD30 monoclonal antibody based therapy. We discuss the immunohistochemistry based approach and the possible diagnostic pitfalls in such cases. The inclusion of markers of plasmablastic differentiation in the diagnostic panel of large cell lymphomas is essential to avoid misclassification of these rare lymphomas.
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PMID:Plasmablastic lymphoma of bone marrow: Report of a rare case and immunohistochemistry based approach to the diagnosis. 3070 70

Plasmablastic lymphoma (PBL) and plasmablastic plasma cell myeloma (PCM) have many overlapping characteristics. Clinical correlation can help make the distinction between the two entities. Human immunodeficiency virus- (HIV-) negative PBL is a rare disease, making the diagnosis more challenging. While there is no standard of care for PBL, current recommendations include dose-adjusted EPOCH (etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone), with or without bortezomib. We report an aggressive case of HIV-negative plasmablastic lymphoma and discuss the challenge in establishing a diagnosis. We review the literature regarding this disease and current recommendations for treatment.
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PMID:A Rare Presentation of HIV-Negative Plasmablastic Lymphoma: A Diagnostic Dilemma. 3090 2

Plasmablastic lymphoma (PBL) is a rare, aggressive, diffuse large B-cell lymphoma usually arising in the oral cavity of human immunodeficiency virus (HIV) patients. Here we describe a patient with HIV who presented with cutaneous nodules that were biopsied and found to be positive for PBL, but whose primary source was found to be testicular. This is the first reported case of a patient with HIV who presented with cutaneous nodules that tested positive for PBL, but whose primary source of neoplasm was found to be testicular.
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PMID:A Unique Case of Testicular Plasmablastic Lymphoma in a Patient with Human Immunodeficiency Virus. 3141 Mar 22


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