Waldenström's macroglobulinemia natural history, complications and prognosis: Difference between revisions
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'''Low-risk disease:'''<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref> | |||
:*0-1 adverse prognostic factors excluding age | |||
:*Five-year survival rate=87% | |||
'''Intermediate-risk disease:''' | |||
:*2 adverse prognostic factors or age | |||
:*Five-year survival rate=68% | |||
'''High-risk disease:''' | |||
:*>2 adverse prognostic factors | |||
:*Five-year survival rate = 36% | |||
==References== | ==References== | ||
Revision as of 15:23, 11 November 2015
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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
Natural History, Complications and Prognosis
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
Common complications
- Hyperviscosity syndrome:[1]
- Most common complication that occurs due to accumulation of excessive monoclonal IgM protein.
- 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
- Venousthromboembolism:
- 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 due to gastrointestinal involvement
- Bleeding manifestations due to dysfunction of platelet, coagulation factor, and fibrinogen
Rare complications
- 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 78 months.[5]
Favorable prognostic factor
- Nodular type of bone marrow involvement
Adverse prognostic factor
- Age older than 65 years
- Hemoglobin < 10 g/dL
- Albumin level < 4.0 g/dL
- Elevated beta-2-microglobulin level
- Elevated Paraprotein concentration
Low-risk disease:[1]
- 0-1 adverse prognostic factors excluding age
- Five-year survival rate=87%
Intermediate-risk disease:
- 2 adverse prognostic factors or age
- Five-year survival rate=68%
High-risk disease:
- >2 adverse prognostic factors
- Five-year survival rate = 36%
References
- ↑ 1.0 1.1 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