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FIGURE 2. RISK STRATIFICATION AND PROGNOSIS ACCORDING TO THE IPSSWM1

An infographic depicting risk stratification and prognosis according to the IPSSWM, including: High risk ≥3 risk factors Intermediate risk Age or 2 risk factors Low risk 0–1 risk factors (except age) IPSSWM risk factors Age >65 years Hb ≤11.5 g/dL Platelet count ≤100x109/L Beta-2 microglobulin >3 mg/L IgM >70 g/L

IPSSWM=International Prognostic Scoring System for WM; IgM=immunoglobulin M; Hb=hemoglobin.
Adapted from Morel P, et al.

ADDITIONAL TESTS

Additional work-up is often based on symptomatic presentation and laboratory findings that can indicate the need for treatment initiation (Table 1).2

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TABLE 1. SELECTED CLINICALLY INDICATED DIAGNOSTIC WORK-UP2,3

Symptom / clinical presentation Test
Older adults Frailty assessment
Hyperviscosity syndrome (symptoms include headaches, visual disturbances, confusion, vertigo, shortness of breath, epistaxis) Serum viscosity
Retinal examination
Anemia with hemolysis Cryoglobulins
Cold agglutinin titre
Unexpected clinical bleeding or bruising von Willebrand disease (VWD) testing
Sensory peripheral neuropathy Myelin-associated glycoprotein antibodies (serum anti-MAGs)
Consider specialist neurological evaluation
Motor neuropathy Consider anti-ganglioside M1 (GM1) antibodies
Amyloidosis should be suspected in patients presenting with: nephrotic syndrome, axonal neuropathy, or unexplained cardiac problems Fat biopsy and/or evaluation of the bone marrow biopsy with Congo red

Adapted with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Waldenström Macroglobulinemia/Lymphoplasmatic Lymphoma V.2.2022 – December 7, 2021 © 2022 National Comprehensive Cancer Network, Inc. All rights reserved. The NCCN Guidelines® and illustrations herein may not be reproduced in any form for any purpose without the express written permission of the NCCN. To view the most recent and complete version of the NCCN Guidelines, go online to NCCN.org. The NCCN Guidelines are a work in progress that may be refined as often as new significant data becomes available. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.

TO TREAT OR NOT TO TREAT?

Elderly woman talking to a healthcare professional

According to the National Comprehensive Cancer Network® (NCCN®), treatment should be initiated only in WM patients who are symptomatic, and when it has been confirmed that their symptoms are not related to or caused by comorbidities.2

Studies have shown that patients with low Hb levels, high lymphoplasmacytic cell infiltration, IgM flare and high beta-2 microglobulin levels may be at higher risk for development of symptomatic WM.4,5 However patients may be asymptomatic for 5–10 years and can be managed with a “watch and wait” approach, without treatment.4

High level of monoclonal IgM alone should not be considered an indication to start treatment.2

Symptomatic hyperviscosity requires immediate disease control. Plasmapheresis should be used immediately in such patients, followed by the initiation of suitable systemic treatment as soon as possible.2

For the most common indications for treatment initiation, visit the “I can identify” page.