title: Human immunodeficiency virus-associated thrombotic microangiopathies: clinical characteristics and outcome according to ADAMTS13 activity.
abstract: Human immunodeficiency virus (HIV) infection is a risk factor for thrombotic microangiopathy (TMA). We sought whether a severe deficiency in ADAMTS13, the enzyme specifically involved in the cleavage of von Willebrand factor, was associated with specific presenting features and outcome in HIV-associated TMA. In this prospective, multicentre, case-control study, 29 patients of 236 in the French Network on TMA had an HIV-associated TMA. Seventeen patients with severe ADAMTS13 deficiency (ADAMTS13 <5% HIV(+) group) were compared to 12 patients with a detectable ADAMTS13 activity (ADAMTS13 >or=5% HIV(+) group). HIV(+) patients were also compared to 62 patients with idiopathic TMA, either with (45 patients, ADAMTS13 <5% idiopathic group) or without (17 patients, ADAMTS13 >or=5% idiopathic group) severe ADAMTS13 deficiency. ADAMTS13 <5% HIV(+) patients had less AIDS-related complications than ADAMTS13 >or=5% HIV(+) patients (23.5% versus 91.6%, respectively, P = 0.0005) and their median CD4(+) T cell count was higher (P = 0.05). TMA-associated death rate was higher in ADAMTS13 >or=5% HIV(+) patients than in ADAMTS13 <5% HIV(+) patients (50% versus 11.7%, respectively, P = 0.04). In ADAMTS13 <5% patients, TMA-associated death rate was comparable between HIV(+) and idiopathic patients (15.5% in idiopathic patients, P-value was non-significant). By contrast, TMA-associated death rate in ADAMTS13 >or=5% HIV(+) patients was higher than in idiopathic patients (11.7% in idiopathic patients, P = 0.04). In conclusion, HIV-associated TMA with severe ADAMTS13 deficiency have less AIDS-related complications and a higher CD4(+) T cell count. TMA prognosis is better and comparable to this of idiopathic forms.
authors.affiliation: Service d'Hématologie et de Thérapie Cellulaire, AP-HP, Hôpital Saint-Antoine and UPMC Université Paris 06, Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Malak
authors.forename: M
authors.initials: M
authors.lastname: Wolf
authors.forename: G A
authors.initials: GA
authors.lastname: Millot
authors.forename: E
authors.initials: E
authors.lastname: Mariotte
authors.forename: A
authors.initials: A
authors.lastname: Veyradier
authors.forename: J-L
authors.initials: JL
authors.lastname: Meynard
authors.forename: J-M
authors.initials: JM
authors.lastname: Korach
authors.forename: S
authors.initials: S
authors.lastname: Malot
authors.forename: A
authors.initials: A
authors.lastname: Bussel
authors.forename: E
authors.initials: E
authors.lastname: Azoulay
authors.forename: E
authors.initials: E
authors.lastname: Boulanger
authors.forename: L
authors.initials: L
authors.lastname: Galicier
authors.forename: E
authors.initials: E
authors.lastname: Devaux
authors.forename: V
authors.initials: V
authors.lastname: Eschwège
authors.forename: S
authors.initials: S
authors.lastname: Gallien
authors.forename: C
authors.initials: C
authors.lastname: Adrie
authors.forename: B
authors.initials: B
authors.lastname: Schlemmer
authors.forename: E
authors.initials: E
authors.lastname: Rondeau
authors.forename: P
authors.initials: P
authors.lastname: Coppo
mesh_terms: D051722:ADAM Proteins / Q000502:physiology*; D000071120:ADAMTS13 Protein; D000163:Acquired Immunodeficiency Syndrome / Q000150:complications*; D000328:Adult; D018791:CD4 Lymphocyte Count; D016022:Case-Control Studies; D003643:Death; D005260:Female; D005602:France; D006678:HIV; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology*; D014841:von Willebrand Factor / Q000502:physiology*
chemical_list: D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human