title: Cancer awareness in atypical thrombotic microangiopathies.
abstract: OBJECTIVE
  To specify the clinical and biological characteristics of thrombotic microangiopathies (TMAs) associated with a recent diagnosis of cancer. PATIENTS AND Methods. Multicenter study involving 14 national centers. Cross-sectional analysis of 20 patients with cancer-associated TMAs included in our national registry from October 2000 to July 2007. Patients were also compared with 134 adult patients with an acquired idiopathic TMA by univariate analysis.
  
  
  RESULTS
  Patients with a cancer-associated TMA typically displayed severe weight loss, dyspnea, bone pain, as well as disseminated intravascular coagulopathy and massive erythromyelemia (75%, 55%, 50%, 41%, and 85% of cases, respectively). By contrast, these features were observed with a much lower incidence in patients with an idiopathic TMA (8.9%, 19.7%, 0.8%, 7.1%, and 17.5%, respectively). Moreover, median platelet count was higher (48 x 10(9)/l; range, 21-73 x 10(9)/l versus 19 x 10(9)/l; range, 10-38 x 10(9)/l, respectively) and median serum creatinine level was lower (74 microM [range, 68-102] versus 113 microM [range, 80-225], respectively). The activity of the specific von Willebrand factor-cleaving proteinase ADAMTS13 was detectable in 14/17 studied patients. Platelet count improvement was observed in only seven patients and paralleled the response to chemotherapy. Prognosis of patients with cancer-associated TMAs was very poor, with a 30-day and 2-year mortality rate of 50% and 95%, respectively.
  
  
  CONCLUSIONS
  Cancer-associated TMAs display specific features at onset that should prompt investigation of an underlying disseminated malignancy. In this context, chemotherapy rather than plasma is mandatory since TMA prognosis parallels that of cancer.
authors.affiliation: Service d'Hématologie et de Thérapie Cellulaire, Hôpital Saint-Antoine, UPMC Univ Paris 06, France.
authors.forename: Lucie
authors.initials: L
authors.lastname: Oberic
authors.forename: Marc
authors.initials: M
authors.lastname: Buffet
authors.forename: Mickael
authors.initials: M
authors.lastname: Schwarzinger
authors.forename: Agnès
authors.initials: A
authors.lastname: Veyradier
authors.forename: Karine
authors.initials: K
authors.lastname: Clabault
authors.forename: Sandrine
authors.initials: S
authors.lastname: Malot
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.forename: Dominique
authors.initials: D
authors.lastname: Valla
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.forename: Leila
authors.initials: L
authors.lastname: Bengrine-Lefèvre
authors.forename: Norbert-Claude
authors.initials: NC
authors.lastname: Gorin
authors.forename: Paul
authors.initials: P
authors.lastname: Coppo
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000368:Aged; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000097:blood / Q000150:complications*; D011446:Prospective Studies; D057049:Thrombotic Microangiopathies / Q000097:blood / Q000150:complications* / Q000201:enzymology; D016896:Treatment Outcome; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human