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β 2 -Microglobulin is the major prognostic factor in multiple myeloma, a known comorbidity of Gaucher disease. We evaluated herein serum β 2 -microglobulin levels of 31 type 1 Gaucher patients; for 8/31 patients, pre- and post-treatment comparisons were made. Thirteen patients (on treatment = 6) had high levels of β 2 -microglobulin, and showed higher chitotriosidase activity and Severity Score Index, and lower concentration of platelets, than patients with normal levels. Levels of β 2 -microglobulin correlated with chitotriosidase activity (ρ = 0.65; p  < 0.01), platelets (ρ = - 0.42; p  = 0.02) and α1- (ρ = 0.43; p  = 0.02) and α2-protein bands (ρ = - 0.40; p  = 0.03). Regarding pre- and post-treatment values, median β 2 -microglobulin levels decreased after treatment (pre- = 2931 ng/mL; post- = 1970 ng/mL; p  < 0.01). Our data suggest that levels of serum β 2 -microglobulin are frequently elevated in type 1 Gaucher patients, correlate with severity of the disease and decrease after treatment.