A comparative analysis of clinical characteristics of patients with paroxysmal nocturnal hemoglobinuria between Asia and Europe/America

Abstract

To accurately analyze the clinical characteristics of paroxysmal nocturnal hemoglobinuria (PNH) in different ethnic backgrounds, we retrieved all retrospective studies on clinical characteristics of PNH with a median follow-up period >60 months published after 2000, analyzed the clinical characteristics of PNH patients in Asia and European/America, and statistically compared enumeration data in these studies. We included 1665 patients in this analysis. The proportion of female patients in Asia was significantly lower than that in Europe/America (P = 0.000). Incidence rates of hemoglobinuria and thromboembolism in Asia were significantly lower than in Europe/America (both P values were 0.000). Within the subgroups of patients with thromboembolism, Asian patients had a higher proportion of arterial thrombosis while Western patients had a higher proportion of abdominal venous thrombosis. Bone marrow failure was not clearly defined in most studies. The proportion of patients with pancytopenia was higher in China than in France (P = 0.048). The total death rates were similar in both ethnic groups (P > 0.05). In Europe/America the major cause of death was thromboembolism and in Asian countries was serious infections. Differences in population characteristics of PNH patients among different ethnic groups indicate the possibility of differential pathogenesis and may be informative for treatment decisions.

Keywords: Cytopenia; Intravascular hemolysis; Paroxysmal nocturnal hemoglobinuria; Thrombosis.