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Thrombosis in paroxysmal nocturnal hemoglobinuria: sites, risks, outcome. An overview
Author(s) -
ZIAKAS P. D.,
POULOU L. S.,
ROKAS G. I.,
BARTZOUDIS D.,
VOULGARELIS M.
Publication year - 2007
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1111/j.1538-7836.2007.02379.x
Subject(s) - medicine , paroxysmal nocturnal hemoglobinuria , thrombosis , population , odds ratio , cohort , fondaparinux , surgery , pediatrics , intensive care medicine , environmental health , venous thromboembolism
MEDLINE Database (1953 to 2006) has been extensively reviewed using the terms PNH, Thrombosis and Paroxysmal Nocturnal Hemoglobinuria, Thrombosis as search criteria; 294 citations were retrieved. Eligible articles were reviews, cohort studies, and case reports providing individual patient data on the site of thrombosis and outcome (death or survival) related to the thrombotic event. Ninety-three articles (nine cohort studies and 84 case reports and reviews of published cases) provided data on 363 PNH cases with thrombosis and outcome in 339 cases. When the same case was reported twice, in a review article and as a case report, only one of the citing articles was included as a data source. When either the site of thrombosis or the outcome was not evident, this case was excluded. Our study population finally consisted of 339 cases of PNH with thrombosis. Treatment options were available in 162 cases and were classified as conventional (n 1⁄4 118) (for those receiving unfractionated heparin, low molecular weight heparin, or warfarin) and interventional modalities (n 1⁄4 44), either to restore blood flow (thrombolysis, anatomic shunts, angioplasty) or reverse coagulation defect (bone marrow transplantation). Odds ratios with their corresponding 95% confidence intervals (CIs) were measured to quantify relative risk of death (RR) in univariate and multivariate logistic regression models. A score chart was developed to facilitate the practical application of the multivariate regression model. The regression coefficients of the significant covariates were rounded off to the proximal 0.5. The value of each predictor (site of thrombosis) has a corresponding score in the chart. The scores are added, resulting in a sum score, which corresponds to a probability according to a logistic transformation. For the significant sites of thrombosis associated with mortality, the population attributable mortality (PAM) was also calculated and depicted. PAM is defined as the proportion of deaths occurring in the total study population (PNH patients with thrombosis) that can be explained by the risk factor (site of thrombosis). Assigned score corresponds to an individual’s probability to die when a thrombosis at this specific site occurs. Instead, PAM refers to the proportion of deaths in the whole study population that is attributed to the particular exposure (specific site of thrombosis). Significance was set to 0.05. The STATA V8 (Stata Corporation, College Station, TX, USA) package was used for statistical analysis.