Waldenström's macroglobulinemia natural history, complications and prognosis: Difference between revisions
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==Complication== | ==Complication== | ||
===Hyperviscosity | 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<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref> | Most common complication that occurs due to accumulation of excessive monoclonal IgM protein<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref> | ||
===Cold | ===Cold Haemagglutinin Disease=== | ||
Occurs due to a cold reactive autoantibody directed against red blood cell antigens that agglutinates red cells at low temperatures | 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=== | ===Cryoglobulinemia=== | ||
1% of patients with | 1% of patients with waldenström's macroglobulinemia have immunoglobulin that precipitate on cooling. | ||
Type II cryoglobulinaemia is associated with | Type II cryoglobulinaemia is associated with hepatitis C virus and presents with [[purpura]], skin ulceration, peripheral neuropathy, [[arthralgia]] and [[glomerulonephritis]]. | ||
===Peripheral | ===Peripheral Neuropathy=== | ||
Can be due to activity of the monoclonal IgM to anti-myelin-associated glycoprotein | Can be due to activity of the monoclonal IgM to anti-myelin-associated glycoprotein | ||
===Venous | ===Venous Thromboembolism=== | ||
Increased risk has been associated with | Increased risk has been associated with waldenström's macroglobulinemia | ||
===Primary | ===Primary Amyloidosis=== | ||
Presents with amyloidosis of the heart, kidney, liver, lungs, and joints<ref name="pa">{{cite journal |vauthors=Zimmermann I, Gloor HJ, Rüttimann S |title=[General AL-amyloidosis: a rare complication in Waldenstrom macroglobulinemia] |language=German |journal=Praxis (Bern 1994) |volume=90 |issue=47 |pages=2050–5 |year=2001 |pmid=11763619 |doi= |url=}}</ref> | Presents with amyloidosis of the heart, kidney, liver, lungs, and joints<ref name="pa">{{cite journal |vauthors=Zimmermann I, Gloor HJ, Rüttimann S |title=[General AL-amyloidosis: a rare complication in Waldenstrom macroglobulinemia] |language=German |journal=Praxis (Bern 1994) |volume=90 |issue=47 |pages=2050–5 |year=2001 |pmid=11763619 |doi= |url=}}</ref> | ||
===Heart | ===Heart Failure=== | ||
Hyperviscosity syndrome and anemia occuring concurrently can cause plasma volume expansion precipitating heart failure.<ref name="HF">{{cite journal |vauthors=Radhakrishnan S, Krishnaswamy M, Bose SC |title=Waldenstrom's macroglobulinaemia, presenting as refractory congestive heart failure |journal=J Assoc Physicians India |volume=26 |issue=8 |pages=754–7 |year=1978 |pmid=103877 |doi= |url=}}</ref> | Hyperviscosity syndrome and anemia occuring concurrently can cause plasma volume expansion precipitating heart failure.<ref name="HF">{{cite journal |vauthors=Radhakrishnan S, Krishnaswamy M, Bose SC |title=Waldenstrom's macroglobulinaemia, presenting as refractory congestive heart failure |journal=J Assoc Physicians India |volume=26 |issue=8 |pages=754–7 |year=1978 |pmid=103877 |doi= |url=}}</ref> | ||
===Diarrhea and | ===Diarrhea and Malabsorption=== | ||
Gastrointestinal involvement may occur in a small number of patients. | |||
===Bleeding | ===Bleeding Manifestations=== | ||
Dysfunction of platelet, coagulation factor, and fibrinogen can occur in few patients with | Dysfunction of platelet, coagulation factor, and fibrinogen can occur in few patients with waldenström's macroglobulinemia | ||
===Richter's | ===Richter's Transformation=== | ||
Also Known as | Also Known as large cell transformation<ref name="iwmf">Waldenström's macroglobulinemia. International Waldenström's macroglobulinemia Foundation (2015) https://en.wikipedia.org/wiki/Medulloblastoma Accessed on September 25, 2015</ref> | ||
===Bing-Neel Syndrome=== | ===Bing-Neel Syndrome=== | ||
Central | Central nervous system [[Lymphoma]]<ref name="bns">{{cite journal |vauthors=Banwait R, O'Regan K, Campigotto F, Harris B, Yarar D, Bagshaw M, Leleu X, Leduc R, Ramaiya N, Weller E, Ghobrial IM |title=The role of 18F-FDG PET/CT imaging in Waldenstrom macroglobulinemia |journal=Am. J. Hematol. |volume=86 |issue=7 |pages=567–72 |year=2011 |pmid=21681781 |doi=10.1002/ajh.22044 |url=}}</ref> | ||
==Prognosis== | ==Prognosis== | ||
Revision as of 06:05, 3 January 2016
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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.[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. 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
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 factor
International Prognostic Scoring System for Waldenstrom Macroglobulinemia uses following 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.