Waldenström’s Macroglobulinemia (WM) is an indolent disease with variable clinical presentation and heterogeneous prognosis. A robust and validated staging system was developed a decade ago, however, since then the treatment of the disease has changed. A revised staging system may better capture the prognosis of WM patients in the chemoimmunotherapy era.
We initially developed the revised system based on data from 492 patients included in the database of the Greek Myeloma Study Group with at least 3 years and a median of 7 years of follow up. An independent validation cohort included 229 symptomatic patients treated in institutions in France and Italy.
we identified that age (≤65 years vs 66-75 vs ≥ 76 years), b2-microglobulin ≥4 mg/L, serum albumin <3.5 gr/dl and LDH ≥250 IU/L can be used to stratify patients in 5 different prognostic groups and identify a very low risk group as well as a very high-risk group. The revised ISSWM identified groups with different outcomes interdependent of age, type of primary therapy or reason to initiate therapy. This simple staging system was further validated in an independent cohort.
A revised IPSSWM may improve patient risk stratification, is easily available and may be used in the everyday practice to provide prognostic information.
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All authors have declared no conflicts of interest.