Lymphoplasmacytic lymphoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Overview

If left untreated, patients with asymptomatic disease may progress to develop fatigue, weight loss, peripheral neuropathy and other symptoms of the disease. Common complications of lymphoplasmacytic lymphoma include: hyperviscosity syndrome, cold haemagglutinin disease, cryoglobulinemia, peripheral neuropathy, primary amyloidosis, renal insufficiency, malabsorptive diarrhea, and visual abnormalities. Prognosis varies depending on the various factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease.

Natural History, Complications, and Prognosis

Natural History:

Initial symptoms

Complications

Late and rare severe complications

Summary of natural history and complications of lymphoplasmacytic lymphoma

 
Initial symptoms:
 
 
 
 
 
 
 
 
Common complications:
 
 
 
 
 
 
 
Late and rare severe complications:
 

Prognosis:

Adverse prognostic factors:

Risk Stratification Criteria

All the above prognostic data has been combined to risk stratify the WM patients and to formulate a standardized scoring system known as the International Prognostic Staging System for Waldenström's Macroglobulinemia (IPSSWM):[23]

Risk factors Score
Age > 65 1
Hemoglobin ≤ 11.5g/dl 1
Platelet ≤ 100,000μl 1
β-microglobulin > 3mg/l 1
IgM > 70g/l 1
International prognostic scoring system for Waldenström macroglobulinemia
Risk group Score 5-year survival Median survival
Low 0-1 (except age) 87% 12 years
Intermediate 2 or age>65 68% 8 years
High ≥3 36% 3.5 years

References

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