Retrospective analysis of prognostic factors for angioimmunoblastic T-cell lymphoma: a multicenter cooperative study in Japan

Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is a major type of peripheral T-cell lymphoma (PTCL).To elucidate the clinicopathologic characteristics and prognosis of AITL in Japan, we retrospectively analyzed 207 patients with AITL. The median patient age was 67 years (range, 34-91 years), with 73% of patients older than 60 years. With a median follow-up of 42 months in surviving patients, 3-year overall survival (OS) was 54% and progression-free survival (PFS) was 38%.The International Prognostic Index (IPI) and the prognostic index for PTCL, not otherwise specified (PIT) were predictive for OS in this analysis. Multivariate analysis found that age older than 60 years, elevated white blood cell (WBC) and IgA levels, the presence of anemia and thrombocytopenia, and extranodal involvement at > 1 site were significant prognostic factors for OS, and IgA, anemia, and mediastinal lymphadenopathy were significant prognostic factors for PFS. A novel prognostic model consisting of the prognostic factors for OS was successfully constructed. In conclusion, IPI and PIT were still useful for prognostication of AITL, and other factors, including those not used in IPI, such as IgA, anemia, WBC count, thrombocytopenia, and mediastinal lymphadenopathy, also significantly affected prognosis. Future investigations for IgA as a unique prognostic factor are warranted.(Blood. 2012;119(12): 2837-2843)

Recent Progress in the Understanding of Angioimmunoblastic T-cell Lymphoma

“…2 The tumor cells express pan-T-cell antigens (i.e., CD3, CD2, and CD5). In contrast, expression of CD7, another pan-T-cell antigen, is less common, 3,4 presumably resulting from hypermethylation of its promoter region. 5 Most cases are positive for CD4 and negative for CD8.…”

“…9 Epstein-Barr virus (EBV)-infected B cells are commonly observed in AITL; EBV-infected B cells were found in 66% of cases in the Japanese retrospective study, 3 9 1 % i n t h e F r e n c h -S w i s s s t u d y, 7 a n d 7 4 % i n t h e COMPLETE study. 8 Many reactive CD8-expressing T cells are often present.…”

“…4 In a Japanese multicenter retrospective study, CD3 was positive in 100% of the samples examined, CD4 in 90%, CD5 in 95%, and CD7 in 28%. 3 Notably, cell-surface CD3 expression is frequently negative in AITL tumor cells. 4,6 In addition, the tumor cells frequently express distinct markers that are expressed by follicular helper T (TFH) cells; in the Japanese multicenter study mentioned above, 3 CD279/PD-1 was positive in 62% of cases, CD10 in 30%, and CXCL13 in 91%.…”

“…3 Notably, cell-surface CD3 expression is frequently negative in AITL tumor cells. 4,6 In addition, the tumor cells frequently express distinct markers that are expressed by follicular helper T (TFH) cells; in the Japanese multicenter study mentioned above, 3 CD279/PD-1 was positive in 62% of cases, CD10 in 30%, and CXCL13 in 91%. CD279/PD-1 was positive in 100%, CD10 in 89%, BCL6 in 91%, CXCL13 in 96%, and ICOS in 98% in a French-Swiss multicenter retrospective study, 7 while CD279/ PD-1 was positive in 95%, CD10 in 66%, and CXCL13 in 84% in the Comprehensive Oncology Measures of Peripheral T-cell Lymphoma (COMPLETE) study, a large prospective cohort study of newly diagnosed peripheral T-cell lymphoma (PTCL) patients in the USA.…”

“…2 As mentioned above, the cells are often EBVpositive (66% -86%), 3,66,67 which may contribute to their clonal expansion.…”