Waldenström's macroglobulinemia natural history, complications and prognosis
|
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, 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
- 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
- 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
- Increased risk has been associated with Waldenström's macroglobulinemia
- 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
Risk stratification
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 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