Angioimmunoblastic T-cell lymphoma in Taiwan reveals worse progression-free survival for RHOA G17V mutated subtype

• Published in: Leukemia & lymphoma Vol. 61; no. 5; pp. 1108 - 1118
• Main Authors: Hsu, Ya-Ting, Wang, Yu-Chu, Chen, Ruo-Yu, Hung, Liang-Yi, Li, Sin-Syue, Yen, Chi-Chieh, Chen, Tsai-Yun, Medeiros, L. Jeffrey, Chang, Kung-Chao
• Format: Journal Article
• Language: English
• Published: United States Taylor & Francis 15.04.2020
• Subjects: Angioimmunoblastic T-cell lymphoma (AITL); clinical features; Female; Humans; Immunoblastic Lymphadenopathy - diagnosis; Immunoblastic Lymphadenopathy - genetics; Lymphoma, T-Cell - diagnosis; Lymphoma, T-Cell - genetics; Male; Middle Aged; next generation sequencing; prognosis; Progression-Free Survival; Retrospective Studies; rhoA GTP-Binding Protein - genetics; rhoA GTP-Binding Protein - metabolism; ROHA G17V mutation; Taiwan - epidemiology; Taiwan; ROHA G17V mutation; Angioimmunoblastic T-cell lymphoma (AITL); prognosis; next generation sequencing; clinical features
• ISSN: 1042-8194; 1029-2403
• DOI: 10.1080/10428194.2019.1702179

Angioimmunoblastic T-cell lymphoma (AITL) carries genetic mutations of TET2, RHOA, and IDH2, but the prognostic impact of these mutations is not widely investigated. Although one study shows no difference in overall survival between patients with or without RHOA G17V mutation, a poor performance status is associated with RHOA G17V-mutated AITL, which is an independent adverse factor. We retrospectively investigated the prognostic impact of RHOA G17V mutation in AITL patients. A total of 31 cases were enrolled (male-to-female, 2.1; mean age: 62.8 years). RHOA G17V mutation was analyzed by deep sequencing. We found that in contrast to RHOA-wild type, patients with RHOA G17V-mutated AITL more frequently had B symptoms (p = .035), stronger PD1 expression (p = .045), ≥3 TFH markers (p = .011), higher blood vessel density (p<.001), and poorer progression-free survival (p = .046). These results support a role for RHOA genetic testing in AITL patients as ROHA G17V mutation carries a worse prognosis, probably associated with B symptoms and stage IV disease.