title: Elevated D-dimer level in the differential diagnosis of venous malformations.
abstract: OBJECTIVE
  To evaluate if elevated D-dimer level is specific for venous malformations (VMs) and thus useful for differential diagnosis, which can be problematic even in specialized interdisciplinary centers. Localized intravascular coagulopathy, characterized by elevated D-dimer levels, has been observed in approximately 40% of patients with VMs.
  
  
  METHODS
  Prospective convenience sample accrued from 2 interdisciplinary sites.
  
  
  METHODS
  Two interdisciplinary centers for vascular anomalies in Brussels, Belgium, and Caen, France
  
  
  METHODS
  The study population comprised 280 patients with clinical data, Doppler ultrasonograms (for 251 patients), and coagulation parameter measurements. Main Outcome Measure Measurement of D-dimer levels.
  
  
  RESULTS
  A VM was diagnosed in 195 of 280 patients (69.6%), and 83 of them had elevated D-dimer levels; the sensitivity of D-dimer dosage was 42.6% (95% confidence interval, 35.6%-49.5%). Among the 85 patients without VM, D-dimer levels were elevated only in 3 patients; the specificity of the dosage was 96.5% (95% confidence interval, 92.5%-100%).
  
  
  CONCLUSIONS
  Elevated D-dimer level is highly specific for VMs (pure, combined, or syndromic), and therefore this easy and inexpensive biomarker test should become part of the clinical evaluation of vascular anomalies. It can detect hidden VMs and help differentiate glomuvenous malformation (normal D-dimer levels) from other multifocal venous lesions. Elevated D-dimer level also differentiates a VM from a lymphatic malformation. Moreover, slow-flow Klippel-Trenaunay syndrome (capillaro-lymphatico-venous malformation with limb hypertrophy) can be distinguished from fast-flow Parkes Weber syndrome (capillary malformation with underlying multiple microfistulas and limb hypertrophy). For these reasons, D-dimer level measurement is a useful complementary tool for diagnosing vascular anomalies in everyday practice.
authors.affiliation: Department of Dermatology, Université de Caen Basse, Normandie, Centre Hospitalier, Universitaire de Caen, Caen, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Dompmartin
authors.forename: Fanny
authors.initials: F
authors.lastname: Ballieux
authors.forename: Pascal
authors.initials: P
authors.lastname: Thibon
authors.forename: Agnès
authors.initials: A
authors.lastname: Lequerrec
authors.forename: Cédric
authors.initials: C
authors.lastname: Hermans
authors.forename: Philippe
authors.initials: P
authors.lastname: Clapuyt
authors.forename: Marie-Thérèse
authors.initials: MT
authors.lastname: Barrellier
authors.forename: Franck
authors.initials: F
authors.lastname: Hammer
authors.forename: Daniel
authors.initials: D
authors.lastname: Labbé
authors.forename: Miikka
authors.initials: M
authors.lastname: Vikkula
authors.forename: Laurence M
authors.initials: LM
authors.lastname: Boon
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000704:Analysis of Variance; D001165:Arteriovenous Malformations / Q000097:blood / Q000175:diagnosis* / Q000000981:diagnostic imaging; D001530:Belgium; D015415:Biomarkers / Q000097:blood; D001778:Blood Coagulation Disorders / Q000097:blood / Q000175:diagnosis; D002196:Capillaries / Q000002:abnormalities*; D016001:Confidence Intervals; D003937:Diagnosis, Differential; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis*; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000097:blood / Q000175:diagnosis; D008206:Lymphatic Diseases / Q000097:blood / Q000175:diagnosis; D008297:Male; D018329:Nevus, Blue / Q000097:blood / Q000175:diagnosis; D011336:Probability; D011446:Prospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D012737:Sex Factors; D018608:Ultrasonography, Doppler; D054079:Vascular Malformations / Q000175:diagnosis; D014680:Veins / Q000002:abnormalities*; D055815:Young Adult
chemical_list: D015415:Biomarkers; D005338:Fibrin Fibrinogen Degradation Products; C036309:fibrin fragment D