Cancer awareness in atypical thrombotic microangiopathies.
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.
Oberic
Lucie
L
Service d'Hématologie et de Thérapie Cellulaire, Hôpital Saint-Antoine, UPMC Univ Paris 06, France.
Buffet
Marc
M
Schwarzinger
Mickael
M
Veyradier
Agnès
A
Clabault
Karine
K
Malot
Sandrine
S
Schleinitz
Nicolas
N
Valla
Dominique
D
Galicier
Lionel
L
Bengrine-Lefèvre
Leila
L
Gorin
Norbert-Claude
NC
Coppo
Paul
P
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
D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human