Paroxysmal Nocturnal Haemoglobinuria (PNH) results in a marked thrombophilic state by unknown mechanisms. Geographic differences in thrombosis incidence in PNH have been observed. We have reviewed 64 patients with "Classic PNH" from a single institution in order to determine the rate of thrombosis in different ethnic groups. When we compared African-Americans (n=11) and Latin-Americans (n=8) with other patients (n=45), we found that African-American and Latin-American patients are at increased risk [Hazard ratio 3.66 (p=0.005) and 3.52, (p=0.035) respectively by Cox regression]. Our data also suggest that this difference in the rate of thrombosis has an impact on length of survival. These findings demonstrate that ethnicity is a risk factor for thrombosis in PNH and have implications for decision-making regarding the management of these patients, including the prevention of thrombosis.
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Thaler et al. (2004) studied this question.