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Cancer Awareness in Atypical Thrombotic Microangiopathies

^aService d'Hématologie et de Thérapie Cellulaire, Hôpital Saint-Antoine, Université Pierre et Marie Curie (UPMC), Paris, France; ^bInstitut National de la Santé et de la Recherche Médicale (INSERM) UMR-S 707, Faculté de Médecine Saint-Antoine, Paris, France; ^cService d'Hématologie Biologique, AP-HP, Hôpital Antoine Béclère, Clamart, et Université Paris-Sud 11, Le Kremlin-Bicêtre, France; ^dService de Réanimation Médicale, Hôpital Charles Nicolle, Rouen, France; ^eService de Médecine Interne, Hôpital de la Conception, Marseille, France; ^fService d'Hépatologie, AP-HP, Hôpital Beaujon, Paris, France; ^gService d'Immunopathologie, AP-HP, Hôpital Saint-Louis, Paris, France; ^hService d'Oncologie Médicale, Assistance Publique–Hôpitaux de Paris (AP-HP), Hôpital Saint-Antoine, Université Pierre et Marie Curie (UPMC), Paris, France

Key Words. Cancer • ADAMTS13 • Thrombotic microangiopathy • Thrombotic thrombocytopenic purpura • Hemolytic uremic syndrome

Correspondence: Correspondence: Paul Coppo, M.D., Ph.D., Service d'Hématologie et de Thérapie Cellulaire, Hôpital Saint-Antoine, UPMC Univ Paris 06, 184 rue du Faubourg Saint-Antoine, Assistance Publique–Hôpitaux de Paris, 75012 Paris, France. Telephone: 00 33 1 49 28 26 21; Fax: 00 33 1 49 28 32 00; e-mail: paulcoppo{at}aol.com

Received April 2, 2009; accepted for publication June 25, 2009.

Disclosures: Lucie Oberic: None; Marc Buffet: None; Mickael Schwarzinger: None; Agnès Veyradier: None; Karine Clabault: None; Sandrine Malot: None; Nicolas Schleinitz: None; Dominique Valla: None; Lionel Galicier: None; Leila Bengrine-Lefèvre: None; Norbert-Claude Gorin: None; Paul Coppo: None.

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 10⁹/l; range, 21–73 x 10⁹/l versus 19 x 10⁹/l; range, 10–38 x 10⁹/l, respectively) and median serum creatinine level was lower (74 µM [range, 68–102] versus 113 µM [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.

Conclusion. 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.