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 macroglobulinemia may progress to develop fatigue, weight loss, peripheral neuropathy and other symptoms of the disease. Common complications of Waldenström 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 various factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease.[1]
Natural History, Complications, and Prognosis
Natural History
- The symptoms of Waldenström macroglobulinemia usually develop in the seventh and eighth decade of life, and start with symptoms such as fatigue, unexplained weight loss, peripheral neuropathy, shortness of breath, purpura, raynaud's phenomenon, and vision problems.[2]
Complications
- Common complications of Waldenström macroglobulinemia include:[1]
- Hyperviscosity syndrome
- Cold haemagglutinin disease
- Cryoglobulinemia
- Peripheral neuropathy
- Venous thromboembolism
- Primary amyloidosis
- Diarrhea and malabsorption
- Bleeding diathesis
- Richter's transformation
- Bing-Neel syndrome
Hyperviscosity Syndrome
- Most common complication that occurs due to accumulation of excessive monoclonal IgM protein
- Symptoms include:
- Vision problem due to retinopathy
- Dizziness
- Cardiac failure
Cold Haemagglutinin Disease
- Occurs due to cold reactive autoantibody directed against red blood cell antigen that agglutinates red blood cells at low temperatures
- Symptoms include:
- Anemia
- Raynaud phenomenon
Cryoglobulinemia
- 1% of patients with waldenström's macroglobulinemia have immunoglobulin that precipitate on cooling
- Symptoms include:
- Type I: is usually asymptomatic
- Type II: is associated with hepatitis C virus and presents with purpura, skin ulceration, peripheral neuropathy, arthralgia and glomerulonephritis.
Peripheral Neuropathy
- Can be due to anti-myelin-associated glycoprotein
- Symptoms include:
- Tremor
- [Ataxia]]
- Loss of vibratory sensation
Less common complications that can develop as a result of waldenström's macroglobulinemia are:
Venous Thromboembolism
- Increased risk has been associated with waldenström's macroglobulinemia
Primary Amyloidosis
- Presents with amyloidosis of the heart, kidney, liver, lungs, and joints[3]
Diarrhea and Malabsorption
- Gastrointestinal involvement may occur in a small number of patients.
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
Prognosis
The median survival of patients with waldenström macroglobulinemia varies from 5 to 11 years.[6]
Favorable prognostic factor:
- Nodular type of bone marrow involvement
Adverse prognostic factors:
- Age > 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
- ↑ Wang H, Chen Y, Li F, Delasalle K, Wang J, Alexanian R; et al. (2012). "Temporal and geographic variations of Waldenstrom macroglobulinemia incidence: a large population-based study". Cancer. 118 (15): 3793–800. doi:10.1002/cncr.26627. PMID 22139816.
- ↑ Zimmermann I, Gloor HJ, Rüttimann S (2001). "[General AL-amyloidosis: a rare complication in Waldenstrom macroglobulinemia]". Praxis (Bern 1994) (in German). 90 (47): 2050–5. PMID 11763619.
- ↑ Waldenström's macroglobulinemia. International Waldenström's macroglobulinemia Foundation (2015) https://en.wikipedia.org/wiki/Medulloblastoma Accessed on September 25, 2015
- ↑ Banwait R, O'Regan K, Campigotto F, Harris B, Yarar D, Bagshaw M, Leleu X, Leduc R, Ramaiya N, Weller E, Ghobrial IM (2011). "The role of 18F-FDG PET/CT imaging in Waldenstrom macroglobulinemia". Am. J. Hematol. 86 (7): 567–72. doi:10.1002/ajh.22044. PMID 21681781.
- ↑ Oza A, Rajkumar SV (2015). "Waldenstrom macroglobulinemia: prognosis and management". Blood Cancer J. 5: e296. doi:10.1038/bcj.2015.28. PMC 4382666. PMID 25815903.