Incidence and treatment outcome of aplastic anemia in Taiwan-real-world data from single-institute experience and a nationwide population-based database.

Aplastic anemia (AA) is a rare disease characterized by pancytopenia and bone marrow failure. The incidence of AA tends to be higher in Asia than in the West, but real-world data about AA in Asia remain limited. We aimed to describe the basic data, treatment, and outcome of AA patients from our institute and evaluate the incidence of AA in Taiwan with a nationwide population-based cohort from National Health Insurance Research Database (NHIRD). We identified patients older than 2 years with AA in the Registry of Catastrophic Illness of NHIRD between 2001 and 2010 and excluded patients with any diagnosis suggestive of congenital or secondary bone marrow failure. With a total of 1270 patients, the overall incidence was 5.67 per million people per year, and there was a biphasic age distribution of incidence rate, highest in ≥ 70 years (19.83 per million people per year) and another peak at age 2-9 years (5.26 per million people per year). Overall, the 5-year survival was 60.0%. Hematopoietic stem cell transplantation (HSCT) and anti-thymocyte globulin-based immunosuppressive therapy (IST) were the major first-line treatments in patients younger than 40 years and were linked with good survival. In contrast, the majority of patients older than 60 years were treated with androgen, and the survival was poor. In multivariate analysis, "severe AA," "very severe AA," and "treatment other than HSCT, IST, or androgen" were independent risk factors for inferior survival. In conclusion, the incidence of AA in Taiwan is consistent with nearby Asian countries and is higher than in the West. Advanced age is associated with higher incidence and poorer outcome.

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

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.