Prognosis and long-term outcomes in type I cryoglobulinemia: A multicenter study of 168 patients.
Fiche publication
Date publication
mai 2023
Journal
American journal of hematology
Auteurs
Membres identifiés du Cancéropôle Est :
Pr LIPSKER Dan
Tous les auteurs :
Ghembaza A, Boleto G, Bommelaer M, Karras A, Javaugue V, Bridoux F, Alyanakian MA, Molinier Frenkel V, Ghillani-Dalbin P, Musset L, Barete S, Roosweil D, Choquet S, Le Joncour A, Mirouse A, Lipsker D, Faguer S, Vieira M, Cacoub P, Biard L, Saadoun D,
Lien Pubmed
Résumé
Type I cryoglobulinemia (CG) accounts for 10%-15% of all cryoglobulinemias and are exclusively seen in clonal proliferative hematologic conditions. In this multicenter nationwide cohort study, we analyzed the prognosis and long-term outcomes of 168 patients with type I CG (93 (55.4%) IgM and 75 [44.6%] IgG). Five- and 10-year event-free survivals (EFS) were 26.5% (95% CI 18.2%-38.4%) and 20.8% (95% CI 13.1%-33.1%), respectively. In multivariable analysis, factors associated with poorer EFS were renal involvement (HR: 2.42, 95% CI 1.41-4.17, p = .001) and IgG type I CG (HR: 1.96, 95% CI 1.13-3.33, p = 0.016), regardless of underlying hematological disorders. IgG type I CG patients had higher cumulative incidence of relapse (94.6% [95% CI 57.8%-99.4%] vs. 56.6% [95% CI 36.6%-72.4%], p = .0002) and death at 10 years (35.8% [19.8%-64.6%] vs. 71.3% [54.0%-94.2%], p = .01) as compared to IgM CG, respectively. Overall, complete response of type I CG at 6 months was 38.7%, with no significant difference between Igs isotypes. In conclusion, renal involvement and IgG CG were identified as independent poor prognostic factors of type I CG.
Référence
Am J Hematol. 2023 05 4;: