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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.<ref name="BM"></ref>   
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.<ref name="BM"></ref>   


 
==Natural History==
==Natural History, Complications and Prognosis==
===Natural History===
*Most patients with Waldenström's macroglobulinemia are initially asymptomatic.  
*Most patients with Waldenström's macroglobulinemia are initially asymptomatic.  
*If left untreated, most of the patients will develop symptomatic Waldenström's macroglobulinemia.  
*If left untreated, most of the patients will develop symptomatic Waldenström's macroglobulinemia.  


===Complication===
==Complication==
=====Common complications=====
===Common complications===
:*[[Hyperviscosity syndrome]]:<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref>
*[[Hyperviscosity syndrome]]:<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.
:*Most common complication that occurs due to accumulation of excessive monoclonal IgM protein.
:*Cold haemagglutinin disease:
*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]]
:*Associated with Anemia and [[Raynaud phenomenon]]
:*[[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.
::*Type II cryoglobulinaemia is associated with Hepatitis C virus and presents with [[purpura]], skin ulceration, peripheral neuropathy, [[arthralgia]] and [[glomerulonephritis]].
:*Type II cryoglobulinaemia is associated with Hepatitis C virus and presents with [[purpura]], skin ulceration, peripheral neuropathy, [[arthralgia]] and [[glomerulonephritis]].
:*Peripheral neuropathy:  
*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 thromboembolism]]:
*[[Venous thromboembolism]]:
::*Increased risk has been associated with Waldenström's macroglobulinemia
:*Increased risk has been associated with Waldenström's macroglobulinemia
:*[[Primary amyloidosis]]:
*[[Primary amyloidosis]]:
::* Presents with amyloidosis of the heart, kidney, liver, lungs, and joints<ref name="COMP">Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-overview#a3 Accessed on November 10, 2015</ref>
:* Presents with amyloidosis of the heart, kidney, liver, lungs, and joints<ref name="COMP">Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-overview#a3 Accessed on November 10, 2015</ref>
:*Heart failure:
*[[Heart failure]]:
::*Hyperviscosity syndrome and anemia occuring concurrently can cause plasma volume expansion precipitating heart failure.<ref name="HF">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&sectionRank=3&anchor=H29#H6 Accessed on November 10, 2015</ref>
:*Hyperviscosity syndrome and anemia occuring concurrently can cause plasma volume expansion precipitating heart failure.<ref name="HF">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&sectionRank=3&anchor=H29#H6 Accessed on November 10, 2015</ref>
:*Diarrhea and malabsorption:  
*Diarrhea and malabsorption:  
::*In few patients gastrointestinal involvement can occur.
:*In few patients gastrointestinal involvement can occur.
:*Bleeding manifestations:
*Bleeding manifestations:
::*Dysfunction of platelet, coagulation factor, and fibrinogen can occur in few patients with Waldenström's macroglobulinemia.
:*Dysfunction of platelet, coagulation factor, and fibrinogen can occur in few patients with Waldenström's macroglobulinemia.


=====Rare complications=====
===Rare complications===
:*[[Richter's transformation]] 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>
*[[Richter's transformation]] 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 - Central Nervous System [[Lymphoma]]  
*Bing-Neel Syndrome - Central Nervous System [[Lymphoma]]  


===Prognosis===
==Prognosis==
Patients with Waldenström macroglobulinemia survive for a median of approximately 6 years.<ref name="Prog">Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-overview#a7 Accessed on November 10, 2015</ref>
Patients with Waldenström macroglobulinemia survive for a median of approximately 6 years.<ref name="Prog">Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-overview#a7 Accessed on November 10, 2015</ref>


=====Favorable prognostic factor=====
===Favorable prognostic factor===
:*Nodular type of bone marrow involvement
*Nodular type of bone marrow involvement


=====Adverse prognostic factor=====
===Adverse prognostic factor===
International Prognostic Scoring System for Waldenstrom Macroglobulinemia uses following adverse prognostic factors:
International Prognostic Scoring System for Waldenstrom Macroglobulinemia uses following adverse prognostic factors:
:*Age older than 65 years
*Age older than 65 years
:*Hemoglobin < 11.5 g/dL
*Hemoglobin < 11.5 g/dL
:*Platelet <1,000,000/microliter
*Platelet <1,000,000/microliter
:*Elevated beta-2-microglobulin level
*Elevated beta-2-microglobulin level
:*Elevated Paraprotein concentration >7 g/dL
*Elevated Paraprotein concentration >7 g/dL
 
===Risk stratification===
=====Risk stratification=====
'''Low-risk disease:'''<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref>  
'''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
*0-1 adverse prognostic factors excluding age
:*Five-year survival rate=87%
*Five-year survival rate=87%
'''Intermediate-risk disease:'''
'''Intermediate-risk disease:'''
:*2 adverse prognostic factors or age
*2 adverse prognostic factors or age
:*Five-year survival rate=68%
*Five-year survival rate=68%
'''High-risk disease:'''
'''High-risk disease:'''
:*>2 adverse prognostic factors
*>2 adverse prognostic factors
:*Five-year survival rate = 36%
*Five-year survival rate = 36%


==References==
==References==

Revision as of 15:05, 17 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

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.
  • 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]
  • Hyperviscosity syndrome and anemia occuring concurrently can cause plasma volume expansion precipitating heart failure.[3]
  • Diarrhea and malabsorption:
  • In few patients gastrointestinal involvement can occur.
  • Bleeding manifestations:
  • Dysfunction of platelet, coagulation factor, and fibrinogen can occur in few patients with Waldenström's macroglobulinemia.

Rare complications

Prognosis

Patients with Waldenström macroglobulinemia survive for a median of approximately 6 years.[5]

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 older than 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=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. ↑ 1.0 1.1 1.2 Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015
  2. ↑ Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-overview#a3 Accessed on November 10, 2015
  3. ↑ 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&sectionRank=3&anchor=H29#H6 Accessed on November 10, 2015
  4. ↑ Waldenström's macroglobulinemia. International Waldenström's macroglobulinemia Foundation (2015) https://en.wikipedia.org/wiki/Medulloblastoma Accessed on September 25, 2015
  5. ↑ Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-overview#a7 Accessed on November 10, 2015

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