Waldenström's macroglobulinemia natural history, complications and prognosis: Difference between revisions
| Line 18: | Line 18: | ||
===Cryoglobulinemia=== | ===Cryoglobulinemia=== | ||
*1% of patients with waldenström's macroglobulinemia have immunoglobulin that precipitate on cooling. | *1% of patients with waldenström's macroglobulinemia have immunoglobulin that precipitate on cooling. | ||
*Symptoms include: | *'''Symptoms include''': | ||
:*Type I: is usually asymptomatic | :*Type I: is usually asymptomatic | ||
:*Type II: is associated with hepatitis C virus and presents with [[purpura]], skin ulceration, peripheral neuropathy, [[arthralgia]] and [[glomerulonephritis]]. | :*Type II: is associated with hepatitis C virus and presents with [[purpura]], skin ulceration, peripheral neuropathy, [[arthralgia]] and [[glomerulonephritis]]. | ||
Revision as of 07:52, 5 January 2016
|
Waldenström's macroglobulinemia Microchapters |
|
Differentiating Waldenström's macroglobulinemia from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
Waldenström's macroglobulinemia natural history, complications and prognosis On the Web |
|
American Roentgen Ray Society Images of Waldenström's macroglobulinemia natural history, complications and prognosis |
|
FDA on Waldenström's macroglobulinemia natural history, complications and prognosis |
|
CDC on Waldenström's macroglobulinemia natural history, complications and prognosis |
|
Waldenström's macroglobulinemia natural history, complications and prognosis in the news |
|
Blogs on Waldenström's macroglobulinemia natural history, complications and prognosis |
|
Directions to Hospitals Treating Waldenström's macroglobulinemia |
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.[1]
- Without treatment, the patient will develop symptoms of fatigue, unexplained weight loss, peripheral neuropathy, shortness of breath, purpura, raynaud's phenomenon, and vision problem.
Complication
Complications that can develop as a result of waldenström's macroglobulinemia are:
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.
- 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 activity of the monoclonal IgM to anti-myelin-associated glycoprotein
- Symptoms include:
- Tremor
- Unsteadiness
- Loss of vibratory sensation
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
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
Central nervous system Lymphoma[5]
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 1.3 Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015
- ↑ 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.
- ↑ Radhakrishnan S, Krishnaswamy M, Bose SC (1978). "Waldenstrom's macroglobulinaemia, presenting as refractory congestive heart failure". J Assoc Physicians India. 26 (8): 754–7. PMID 103877.
- ↑ 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.