Analysis of clinicopathological features and prognostic factors of non‑Hodgkin's intravascular large B‑cell lymphoma

Abstract

Non-Hodgkin's intravascular large B-cell lymphoma is a highly invasive extranodal lymphoma. The proliferating tumor cells invade the small vessels and capillaries of different organs. The clinical symptoms are atypical, there is lack of specificity, and the molecular and biological behaviors are not clear, thus, the present study aimed to improve the current understanding of non-Hodgkin's intravascular large B-cell lymphoma (IVL) and provide an accurate basis for clinical treatment and prognosis, by retrospectively analyzing and summarizing the clinicopathological features, immunohistochemical findings and molecular characteristics of 17 patients with IVL. The Kaplan-Meier method and log rank test were implemented to determine survival outcomes. Fisher's exact test was used to determine the association between clinicopathological features and the expression levels of Ki-67, c-Myc, B-cell lymphoma 6 (Bcl-6) and B-cell lymphoma 2 (Bcl-2), while multivariate Cox regression analysis was performed to identify the independent risk factors that affect the survival rates of patients with IVL. P<0.05 was considered to indicate a statistically significant difference. Among the 17 patients with IVL, 13 cases (76.47%) occurred in the adrenal gland and four cases (23.53%) occurred on the skin demonstrated positive IgH gene rearrangement. FISH analysis indicated that cleavage of the c-Myc gene was closely associated with sex, hypertension status and tumor size, while cleavage of the Bcl-6 gene was closely associated with tumor size parameters. Overall, the results suggest that the Ki-67 proliferation index is an independent risk factor for the prognosis (survival time) of patients with IVL.

Intravascular Large B-Cell Lymphoma: A Review with a Focus on the Prognostic Value of Skin Involvement

“…The intravascular cells usually express CD45, CD20, the surface marker of B cells, and CD79a, a B-cell antigen receptor marker [36,37], but are not reactive to CD3 (Figure 2D-F). The cells have a high Ki-67 proliferation index, which was of negative prognostic value in one retrospective case review [38]. The large atypical cells stain positive for Bcl-2 and multiple myeloma 1 (MUM1) and variably for Bcl-6 and CD10.…”

Cutaneous Intravascular Hematolymphoid Entities: A Review

“…In the recent World Health Organization (WHO) classification, IVLBCL was grouped in the large B-cell lymphomas category [31]. The tumor cells of IVLBCL express CD20 (99-100%), Pax5 (100%), CD79a (100%), and other B-cell markers such as CD19, BOB.1, and OCT2 [2,14,16,32]. BCL2 (80-100%) [11], CD10 (13%) [17], and MUM1 (75-100%) positivity [14,16,33], with varied positivity for BCL6 [16,33], were reported.…”

Intravascular Large B-Cell Lymphoma

“…DIAGNOSIS IVLBCL usually occurs in patients between the ages of 34 and 90 years with a variable reported median range of 60 to 70 years. [5][6][7] The age-adjusted incidence within the United States was 0.095 per 1 million people between the years 2000 and 2013. 7 Symptoms at presentation are based on the site of involvement with central nervous system (CNS) and skin being the most common locations.…”

“…4,14 Reports of BCL6 positivity are variable with ranges of 22% to 86%. 6,14,18 When tumor cells are negative for germinal center markers (ie, CD10 and BCL6), MUM1/IRF4 is usually expressed. 4,18 Other markers displaying positivity can include BCL2 and rarely CD23.…”

“…[19][20][21] Ki-67 proliferation indices are frequently high, with expression above 50% in most cases (Table ). 6,21,22 One small case series explored programmed death-ligand 1 (PD-L1) immunoexpression, noting that a subset of IVLBCL cases were highlighted by this marker (45.4%, of n ¼ 11), with potential implications for targeted therapy. 23 Additionally,…”