Stage, age, and EBV status impact outcomes of plasmablastic lymphoma patients: a clinicopathologic analysis of 61 patients

Abstract

BackgroundPlasmablastic lymphoma (PBL) is a rare aggressive neoplasm with lymphoid and plasmacytic differentiation that is commonly associated with immunodeficiency and an unfavorable prognosis. Clinicopathologic features have been largely derived from cases reports and small series with limited outcome analyses.Patients and methodsThe demographic, clinicopathologic features, and clinical outcomes of a cohort of 61 patients with PBL were reviewed and analyzed.ResultsPatients had a median age of 49 years (range 21–83 years) and most (49/61; 80 %) were men. Human immunodeficiency virus (HIV) status was available for 50 patients: 20 were HIV-positive and 30 HIV-negative. Twenty-three patients were immunocompetent. Abdominal/gastrointestinal complaints were the most common presenting symptoms, reported in 14 of 47 (30 %) of patients. At presentation, 24 of 43 (56 %) patients had stage III or IV disease. Epstein-Barr virus (EBV) was detected in 40 of 57 (70 %) cases. MYC rearrangement was identified in 10/15 (67 %) cases assessed, and MYC overexpression was seen in all cases assessed regardless of MYC rearrangement status. HIV-positive patients were significantly younger than those who were HIV-negative (median 42 vs. 58 years; p = 0.006). HIV-positive patients were also significantly more likely to have EBV-positive disease compared with HIV-negative patients (19/19, 100 % vs. 15/29, 52 %; p = 0.002). Patients who received CHOP chemotherapy tended to have better overall survival (OS) compared with those who received hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD) (p = 0.078). HIV status had no impact on OS. Patients with EBV-positive PBL had a better event-free survival (EFS) (p = 0.047) but not OS (p = 0.306). Notably, OS was adversely impacted by age ≥50 years (p = 0.013), stage III or IV disease (p = <0.001), and lymph node involvement (p = 0.008).ConclusionsThe most significant prognostic parameters in patients with PBL are age, stage, and, to a lesser extent, EBV status. In this study, two-thirds of PBL cases assessed were associated with MYC rearrangement and all showed MYC overexpression.Electronic supplementary materialThe online version of this article (doi:10.1186/s13045-015-0163-z) contains supplementary material, which is available to authorized users.

Immune-checkpoint expression in Epstein-Barr virus positive and negative plasmablastic lymphoma: a clinical and pathological study in 82 patients

“…1 It commonly occurs in the context of human immunodeficiency virus (HIV) infection or in association with other contexts of immunodeficiency such as autoimmune diseases, organ transplantation and in the elderly; [2][3][4][5] few studies have identified PL in patients without immunodeficiency. 6,7 Neoplastic cells resemble immunoblasts or plasmablasts with a B-cell terminal differentiation phenotype and they mostly have a non-germinal center (GC)-B subtype profile. [2][3][4][5][6][7][8] These cells constitutively express the plasma cell antigen CD138 (syndecan-1) and often harbor immunoglobulin (Ig) chain restriction with no or weak expression of B-cell mature markers such as CD20, CD79a and PAX5.…”

“…6,7 Neoplastic cells resemble immunoblasts or plasmablasts with a B-cell terminal differentiation phenotype and they mostly have a non-germinal center (GC)-B subtype profile. [2][3][4][5][6][7][8] These cells constitutively express the plasma cell antigen CD138 (syndecan-1) and often harbor immunoglobulin (Ig) chain restriction with no or weak expression of B-cell mature markers such as CD20, CD79a and PAX5.…”

Spontaneous Regression of an Oral Manifestation of Plasmablastic Lymphoma: Literature Review and Commentary about the Phenomena

“…PBL commonly affects extranodal sites, predominantly the oral cavity, gastrointestinal tract and the skin Contrasting, in the present case, our patient is a female patient and had a more advanced age than most seropositive patients. The best prognosis is associated with younger patients, early stage of the disease and no lymph node involved [12].…”

Histologic transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma

“…Plasmablastic lymphoma (PBL) is an aggressive B-cell malignancy, thought to be related to DLBCL, in which the lymphoma cells exhibit morphologic and immunophenotypic features of plasma cell differentiation [46] (Supporting Information Fig. 3).…”