Primary adrenal plasmablastic lymphoma presenting as lymphomatous meningitis – A diagnostic perplexity

Abstract

A rare presentation of primary plasmablastic lymphoma (PBL) of the adrenal gland, as lymphomatous meningitis in a patient living with HIV.

A Case Report of Plasmablastic Lymphoma with Unusual Presentation: How Do Socioeconomic Factors Contribute to Cancer Survival?

“…This immunostaining is consistent with prior studies which demonstrate that the neoplastic cells in PBL are negative for B-cell markers such as CD19, CD20, and PAX-5; most or all neoplastic cells are positive for Ki-67 and about 70% of cases express EBV-encoded RNA (EBER) (Pathak et al, 2021;Castillo et al, 2015;Al Sbihi et al, 2020). There is variable expression for CD38 and CD138 in general, and in most cases is positive but was negative in this patient (Al Sbihi et al, 2020).…”

“…PBL is characterized by plasmablasts, which are lymphoid cells that have lost the characteristic B-cell surface markers and have instead acquired plasma cell surface markers (Nwanwene et al, 2021), its features overlap with myeloma and lymphoma making diagnosis difficult. Hence, PBL cells show plasmacytic differentiation markers-CD38, CD138, MUM1, Blimp1, XBP1, and MYC-with variable expression for CD45, CD79a, EMA, and CD30 (Pathak et al, 2021). Generally, there is no expression of Bcell markers (CD20 and PAX5) (Pathak et al, 2021).…”

“…Hence, PBL cells show plasmacytic differentiation markers-CD38, CD138, MUM1, Blimp1, XBP1, and MYC-with variable expression for CD45, CD79a, EMA, and CD30 (Pathak et al, 2021). Generally, there is no expression of Bcell markers (CD20 and PAX5) (Pathak et al, 2021). Given its rarity and overall dismal prognosis of patients with PBL, there is no established standard of care for patients with this disease (Castillo et al, 2015).…”

“…Small case series have shown that intensive chemotherapies combined with antimyeloma agents, such as Bortezomib and Lenalidomide may be effective in treating PBL (Castillo et al, 2015). The prognosis of patients with PBL is generally poor with a median overall survival of 6-19 months, and there are no clear-cut differences between HIV-positive and HIV-negative patients based on a meta-analysis of 277 patients (Pathak et al, 2021;Morscio et al, 2014) The patient in this case report was treated with R-EPOCH therapy and received two cycles of inpatient chemotherapy with multiple complications. This patient had treatment delays totaling 88 days including 66 days from initial presentation before presenting to our facility, Overall, the patient was in the hospital for 94 days during which he was only able to complete 2 cycles of chemotherapy because of multiple complications including paraplegia, thoracic wound dehiscence, profound bone marrow suppression, infections, clostridium difficile diarrhea, mucositis, esophagitis, and poor nutrition.…”