title: Study of Anticoagulant, Procoagulant, and Fibrinolytic Pathways in Mexican Patients With Paroxysmal Nocturnal Hemoglobinuria.
abstract: BACKGROUND
  In Mexico, the frequency of thromboembolic events associated to paroxysmal nocturnal hemoglobinuria is 3%; a clone size > 50% in granulocytes has been associated with a higher risk of thromboembolic events.
  
  
  METHODS
  Between 2001 and 2012, 40 patients with paroxysmal nocturnal hemoglobinuria were studied. In 12 cases anticoagulant, procoagulant, and fibrinolytic pathways were analyzed.
  
  
  RESULTS
  Only two of 40 patients (5%) developed a thromboembolic event over a 25.5-year follow-up period. From 12 patients, 91.7% had a paroxysmal nocturnal hemoglobinuria clone > 50% in granulocytes and 83.3% a clone > 50 % in monocytes. Five of 12 cases had elevated FV levels and four showed increased FVIII, von Willebrand factor antigen, von Willebrand factor ristocetin cofactor activity and FX. Protein S and protein C were decreased in nine and three patients, respectively. Only antithrombin correlated positively with paroxysmal nocturnal hemoglobinuria clone size in monocytes (p = 0.0442), whereas von Willebrand factor ristocetin cofactor correlated negatively with lactic dehydrogenase levels (p = 0.0186). No statistically significant associations were recorded with all other factors.
  
  
  CONCLUSIONS
  The low frequency of thromboembolic events in Mexican patients could partly be explained by the associations between anticoagulant system (antithrombin) with paroxysmal nocturnal hemoglobinuria monocyte clone size, and procoagulant system (von Willebrand factor ristocetin cofactor) with lactic dehydrogenase levels.
authors.affiliation: Department of Surgery Hematology and Oncology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
authors.forename: Aldo A
authors.initials: AA
authors.lastname: Scherling-Ocampo
authors.affiliation: Department of Surgery Hematology and Oncology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
authors.forename: Ángel G
authors.initials: ÁG
authors.lastname: Vargas-Ruíz
authors.affiliation: Department of Surgery Hematology and Oncology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
authors.forename: Xavier
authors.initials: X
authors.lastname: López-Karpovitch
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000925:Anticoagulants / Q000378:metabolism*; D001779:Blood Coagulation Factors / Q000378:metabolism*; D002648:Child; D015331:Cohort Studies; D005260:Female; D005342:Fibrinolysis / Q000502:physiology; D005500:Follow-Up Studies; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008800:Mexico / Q000453:epidemiology; D008875:Middle Aged; D012189:Retrospective Studies; D013923:Thromboembolism / Q000453:epidemiology* / Q000209:etiology; D055815:Young Adult
chemical_list: D000925:Anticoagulants; D001779:Blood Coagulation Factors