Khwaja, Jahanzaib;
Vos, Josephine MI;
Pluimers, Tessa E;
Japzon, Nicole;
Patel, Aisha;
Salter, Simon;
Kwakernaak, Arjan J;
... D'Sa, Shirley; + view all
(2024)
Clinical and clonal characteristics of monoclonal immunoglobulin M-associated type I cryoglobulinaemia.
British Journal of Haematology
, 204
(1)
pp. 177-185.
10.1111/bjh.19112.
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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.
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