Clinical, pathological and molecular features of plasmablastic lymphoma arising in the gastrointestinal tract: A review and reappraisal

Survival analysis in treated plasmablastic lymphoma patients: a population‐based study

“…Our analysis in the HIV cohort did not demonstrate a significant relation between oral primary location and HIV infection in PBL patients. In addition, similar to recent report by Arora et al, 21 we observed high incidence of primary location in gastrointestinal tract that is more commonly observed in HIV negative PBL patients where it is as frequent as primary oral localization 4,12 . It is unclear whether incidence of primary oral and gastrointestinal locations is changing with the almost universal use of antiretroviral medications or due to better recognition and awareness for common localization of PBL in non‐oral areas and occurrence in HIV negative patients.…”

“…It is considered to be a subtype of large B‐cell lymphoma with plasmablastic morphology and expression of plasma cell markers (CD79a, CD38, CD138 and MUM‐1), but lacking expression of B‐cell markers (CD20, PAX‐5) 3 . The differential diagnosis includes immunoblastic diffuse large B‐cell lymphoma, ALK positive diffuse large B‐cell lymphoma, HHV‐8 positive large B‐cell lymphoma, primary effusion lymphoma, plasmablastic or anaplastic multiple myeloma and undifferentiated carcinoma 3‐5 . Nodal involvement and association with HIV and EBV are typical for PBL but not seen in plasma cell neoplasms 5‐7 …”

Recent advances in upper gastrointestinal lymphomas: molecular updates and diagnostic implications

“…149 The GI tract is the most common site of presentation, after the oral cavity, and~50% of primary GI plasmablastic lymphomas are diagnosed in the stomach or proximal small intestine. [150][151][152][153] Although plasmablastic lymphoma was initially described in HIV+ individuals, the majority of cases nowadays occur in HIV-patients with other causes of immunosuppression or apparently immunocompetent. 151,154,155 Plasmablastic lymphoma is essentially negative for CD45, CD20, and PAX5, negative or variably positive for CD79a, and positive for CD38, CD138, and VS38c, with expression of the transcription factors PRDM1/BLIMP-1, MUM1, and XBP1.…”

“…150,151,153 Approximately half of the cases are EBV+ (EBER+; Epstein-Barr nuclear antigen 1 (EBNA1)+). 150,151,153 MYC rearrangements are found in 50% of the cases, usually with an IG gene as the translocation partner. [151][152][153]156 Recent NGS-based studies have reported recurrent mutations affecting the JAK-STAT (STAT3, JAK1, SOCS1, JAK2, and PIM1), RAS-mitogen-activated protein kinase (MAPK) (NRAS, KRAS, BRAF, and MAP2K1) and Notch (NOTCH1, SPEN, and NCOR2) signalling pathways, providing the genetic basis for new therapeutic interventions.…”

“…Cytoplasmic immunoglobulin is detected in 50–70% of the cases, and CD56 is variably expressed 150,151,153 . Approximately half of the cases are EBV+ (EBER+; Epstein–Barr nuclear antigen 1+) 150,151,153 . MYC rearrangements are found in 50% of the cases, usually with an immunoglobulin gene as the translocation partner 151–153,156 .…”