Prognosis and long‐term outcomes in type I cryoglobulinemia: A multicenter study of 168 patients
Amine Ghembaza, Gonçalo Boleto, Marin Bommelaer, Alexandre Karras, Vincent Javaugue, Frank Bridoux, Marie‐Alexandra Alyanakian, Valérie Molinier‐Frenkel, Pascale Ghillani‐Dalbin, Lucile Musset, Stéphane Barète, Damien Roos‐Weil, Sylvain Choquet, Alexandre Le Joncour, Adrien Mirouse, Dan Lipsker, Stanislas Faguer, Matheus Vieira, P. Cacoub, Lucie Biard, David Saadoun, from the French Cryoglobulinemia Network
Abstract
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.