Prognosis
Waldenström's macroglobulinaemia (WM) is an incurable chronic condition. Median overall survival is approximately 8 years (based on US Surveillance Epidemiology and End Results [SEER] data) though this will have improved significantly with the use of Bruton’s tyrosine kinase inhibitors and other novel agents.[49][54]
Prior to the availability of newer targeted therapies, approximately 50% of patients with WM would die of unrelated causes due to the late age at presentation of the disease.[32] Survival depends on many factors related to disease burden, patient age, and comorbidities.
Prognostic scoring systems for WM
The international prognostic scoring system for Waldenström's macroglobulinaemia (IPSSWM) defines three risk groups (low, intermediate, and high) based on age, haemoglobin level, platelet count, beta-2 microglobulin level, and serum monoclonal immunoglobulin M (IgM) level.[51] See Criteria.
The 5-year overall survival rates for each risk group of the IPSSWM are:
Low risk: 87%
Intermediate risk: 68%
High risk: 36%
A revised version of IPSSWM defines five risk groups (very low, low, intermediate, high, and very high) based on age, beta-2 microglobulin level, lactate dehydrogenase (LDH) level, and albumin level.[45] See Criteria.
The 5-year overall survival rates for each risk group of the revised IPSSWM are:
Very low risk: 95%
Low risk: 86%
Intermediate risk: 78%
High risk: 47%
Very high risk: 36%
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