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, shortness of breath, purpura, raynaud's phenomenon, and vision problems. Common complications of lymphoplasmacytic lymphoma include: hyperviscosity syndrome, cold haemagglutinin disease, cryoglobulinemia, peripheral neuropathy, primary amyloidosis, renal insufficiency, malabsorptive diarrhea, visual abnormalities, congestive heart failure, and schnitzler syndrome. Late and rare severe complications include richter syndrome, and bing-Neel syndrome. Prognosis varies depending on the various factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease. A standardized scoring system known as the International Prognostic Staging System for Waldenström's Macroglobulinemia (IPSSWM) risk stratifies the patients with Waldenstrom's macroglobulinemia.

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):[25]

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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