Waldenström's macroglobulinemia 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: Mirdula Sharma, MBBS [2]
Overview
If left untreated, patients with asymptomatic Waldenström's macroglobulinemia may progress to develop symptomatic disease. Common complications of Waldenström's macroglobulinemia include hyperviscosity syndrome, cold haemagglutinin disease, cryoglobulinemia, peripheral neuropathy, venous thromboembolism, primary amyloidosis, heart failure, malabsorbtive diarrhea, and bleeding manifestations. Prognosis varies depending on the prognostic factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease.[1]
Natural History
- Most patients with Waldenström's macroglobulinemia are initially asymptomatic.
- If left untreated, most of the patients will develop symptomatic Waldenström's macroglobulinemia.
Complication
Hyperviscosity syndrome
Most common complication that occurs due to accumulation of excessive monoclonal IgM protein[1]
Cold haemagglutinin disease
Occurs due to a cold reactive autoantibody directed against red blood cell antigens that agglutinates red cells at low temperatures Associated with Anemia and Raynaud phenomenon
Cryoglobulinemia
1% of patients with Waldenström's macroglobulinemia have immunoglobulin that precipitate on cooling. Type II cryoglobulinaemia is associated with Hepatitis C virus and presents with purpura, skin ulceration, peripheral neuropathy, arthralgia and glomerulonephritis.
Peripheral neuropathy
Can be due to activity of the monoclonal IgM to anti-myelin-associated glycoprotein
Venous thromboembolism
Increased risk has been associated with Waldenström's macroglobulinemia
Primary amyloidosis
Presents with amyloidosis of the heart, kidney, liver, lungs, and joints[2]
Heart failure
Hyperviscosity syndrome and anemia occuring concurrently can cause plasma volume expansion precipitating heart failure.[3]
Diarrhea and malabsorption
In few patients gastrointestinal involvement can occur
Bleeding manifestations
Dysfunction of platelet, coagulation factor, and fibrinogen can occur in few patients with Waldenström's macroglobulinemia
Richter's transformation
Also Known as Large Cell Transformation[4] Bing-Neel Syndrome: Central Nervous System Lymphoma
Prognosis
Patients with Waldenström macroglobulinemia survive for a median of approximately 6 years.[5]
Favorable prognostic factor
- Nodular type of bone marrow involvement
Adverse prognostic factor
International Prognostic Scoring System for Waldenstrom Macroglobulinemia uses following adverse prognostic factors:
- Age older than 65 years
- Hemoglobin < 11.5 g/dL
- Platelet <1,000,000/microliter
- Elevated beta-2-microglobulin level
- Elevated Paraprotein concentration >7 g/dL
Risk stratification
Low-risk disease:[1]
- 0-1 adverse prognostic factors excluding age
- Five-year survival rate is 87%
Intermediate-risk disease:
- 2 adverse prognostic factors or age
- Five-year survival rate is 68%
High-risk disease:
- >2 adverse prognostic factors
- Five-year survival rate is 36%
References
- ↑ 1.0 1.1 1.2 Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015
- ↑ Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-overview#a3 Accessed on November 10, 2015
- ↑ Waldenström's macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=machineLearning&search=Waldenström%27s+macroglobulinemia&selectedTitle=1%7E80§ionRank=3&anchor=H29#H6 Accessed on November 10, 2015
- ↑ Waldenström's macroglobulinemia. International Waldenström's macroglobulinemia Foundation (2015) https://en.wikipedia.org/wiki/Medulloblastoma Accessed on September 25, 2015
- ↑ Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-overview#a7 Accessed on November 10, 2015