Clinical and clonal characteristics of monoclonal immunoglobulin M-associated type I cryoglobulinaemia.
Br J Haematol
; 204(1): 177-185, 2024 01.
Article
in En
| MEDLINE
| ID: mdl-37726004
ABSTRACT
Monoclonal immunoglobulin M-associated type I cryoglobulinaemia is poorly characterised. We screened 534 patients with monoclonal IgM disorders over a 9-year period and identified 134 patients with IgM type I cryoglobulins. Of these, 76% had Waldenström macroglobulinaemia (WM), 5% had other non-Hodgkin lymphoma (NHL) and 19% had IgM monoclonal gammopathy of undetermined significance (MGUS). Clinically relevant IgM-associated disorders (including cold agglutinin disease [CAD], anti-MAG antibodies, amyloidosis and Schnitzler syndrome) coexisted in 31%, more frequently in MGUS versus WM/NHL (72% vs. 22%/29%, p < 0.001). The majority of those with cryoglobulins and coexistent CAD/syndrome had the molecular characteristics of a CAD clone (wild-type MYD88 in 80%). A half of all patients had active manifestations at cryoglobulin detection vasomotor (22%), cutaneous (16%), peripheral neuropathy (22%) and hyperviscosity (9%). 16/134 required treatment for cryoglobulin-related symptoms alone at a median of 38 days (range 6-239) from cryoglobulin detection. At a median follow-up of 3 years (range 0-10), 3-year cryoglobulinaemia-treatment-free survival was 77% (95% CI 68%-84%). Age was the only predictor of overall survival. Predictors of cryoglobulinaemia-related treatment/death were hyperviscosity (HR 73.01; 95% CI 15.62-341.36, p < 0.0001) and cutaneous involvement (HR 2.95; 95% CI 1.13-7.71, p = 0.028). Type I IgM cryoglobulinaemia is more prevalent than previously described in IgM gammopathy and should be actively sought.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Monoclonal Gammopathy of Undetermined Significance
/
Lymphoma, B-Cell
/
Waldenstrom Macroglobulinemia
/
Cryoglobulinemia
Type of study:
Prognostic_studies
/
Risk_factors_studies
Limits:
Humans
Language:
En
Journal:
Br J Haematol
Year:
2024
Document type:
Article
Affiliation country:
Reino Unido