Waldenström's macroglobulinemia natural history, complications and prognosis: Difference between revisions

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==Overview==
==Overview==
If left untreated, patients with asymptomatic Waldenström macroglobulinemia may progress to develop [[fatigue]], [[weight loss]], [[peripheral neuropathy]] and other symptoms of the disease. Common complications of Waldenström macroglobulinemia include: [[hyperviscosity syndrome]], [[Cold agglutinin disease|cold haemagglutinin disease]], [[cryoglobulinemia]], [[peripheral neuropathy]], [[primary amyloidosis]], [[Renal insufficiency|renal insufficiency,]] [[Diarrhea|malabsorbtive diarrhea]], and [[Blurred vision|visual abnormalities]]. [[Prognosis]] varies depending on the various factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease.
If left untreated, patients with asymptomatic Waldenström macroglobulinemia may progress to develop [[fatigue]], [[weight loss]], [[peripheral neuropathy]] and other symptoms of the disease. Common complications of Waldenström macroglobulinemia include: [[hyperviscosity syndrome]], [[Cold agglutinin disease|cold haemagglutinin disease]], [[cryoglobulinemia]], [[peripheral neuropathy]], [[primary amyloidosis]], [[Renal insufficiency|renal insufficiency,]] [[Diarrhea|malabsorbtive diarrhea]], and [[Blurred vision|visual abnormalities]]. [[Prognosis]] varies depending on the various factors involved. A five-year survival rate is 87% for low-risk disease and 36% for high-risk disease.


==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
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*[[Central Nervous system Lymphoma]] ([[Bing-Neel syndrome]]).<ref name="pmid9686227">{{cite journal| author=Civit T, Coulbois S, Baylac F, Taillandier L, Auque J| title=[Waldenström's macroglobulinemia and cerebral lymphoplasmocytic proliferation: Bing and Neel syndrome. Apropos of a new case]. | journal=Neurochirurgie | year= 1997 | volume= 43 | issue= 4 | pages= 245-9 | pmid=9686227 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9686227  }} </ref>
*[[Central Nervous system Lymphoma]] ([[Bing-Neel syndrome]]).<ref name="pmid9686227">{{cite journal| author=Civit T, Coulbois S, Baylac F, Taillandier L, Auque J| title=[Waldenström's macroglobulinemia and cerebral lymphoplasmocytic proliferation: Bing and Neel syndrome. Apropos of a new case]. | journal=Neurochirurgie | year= 1997 | volume= 43 | issue= 4 | pages= 245-9 | pmid=9686227 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9686227  }} </ref>
**The development of Waldenström macroglobulinemia cells in the central nervous system was first described by Drs. Bing and Neel and carries their names as the Bing-Neel syndrome. WM involves CNS in following two forms:
**The development of Waldenström macroglobulinemia cells in the central nervous system was first described by Drs. Bing and Neel and carries their names as the Bing-Neel syndrome. WM involves CNS in following two forms:
***Actual tumor developing in the brain substance causing seizures and paralysis.
***Actual tumor developing in the brain substance causing [[seizures]] and [[paralysis]].
***Tumor cells invading meninges and cranial nerves without causing the actual tumors and with or without CSF cryoglobulinemia,<ref name="pmid19362988">{{cite journal| author=Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH| title=Bing-Neel Syndrome revisited. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 1 | pages= 104-6 | pmid=19362988 | doi=10.3816/CLM.2009.n.028 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19362988  }} </ref> leading to following symptoms:<ref name="pmid19951888">{{cite journal| author=Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG| title=Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 6 | pages= 462-6 | pmid=19951888 | doi=10.3816/CLM.2009.n.091 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19951888  }} </ref>
***Tumor cells invading meninges and cranial nerves without causing the actual tumors and with or without [[CSF]] [[cryoglobulinemia]],<ref name="pmid19362988">{{cite journal| author=Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH| title=Bing-Neel Syndrome revisited. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 1 | pages= 104-6 | pmid=19362988 | doi=10.3816/CLM.2009.n.028 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19362988  }} </ref> leading to following symptoms:<ref name="pmid19951888">{{cite journal| author=Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG| title=Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 6 | pages= 462-6 | pmid=19951888 | doi=10.3816/CLM.2009.n.091 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19951888  }} </ref>
****Headache.
****[[Headache]]
****Confusion.
****Confusion
****Neck stiffness.
****[[Neck stiffness]]
****Sporadic loss of motor function.
****Sporadic loss of motor function  
****Facial paralysis.
****[[Facial paralysis]]
****Drooping eyelid.
****[[Drooping eyelid]]
****Double vision.
****[[Double vision]]
****Difficult swallowing.
****[[Dysphagia|Difficultly swallowing]]
****Visual loss.
****Visual loss
****Hearing loss.
****Hearing loss


=== Prognosis ===
=== Prognosis ===
*Prognosis is generally poor.
*[[Prognosis]] is generally poor.
*The median survival from the time of diagnosis is 6.4 years.<ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
*The median survival from the time of diagnosis is 6.4 years.<ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
*The median disease-specific survival is 11.2 years.<ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
*The median disease-specific survival is 11.2 years.<ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
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*5-year survival rate is 78%.
*5-year survival rate is 78%.
*In the last decade (2001-2010), the median overall survival for all WM groups has improved to just over 8 years compared to 6 years in the previous decade (1991-2000).
*In the last decade (2001-2010), the median overall survival for all WM groups has improved to just over 8 years compared to 6 years in the previous decade (1991-2000).
*After 2000, a 2-fold increased mortality is reported in patients diagnosed with WM when compared with expected population mortality.<ref name="KyleLarson2018">{{cite journal|last1=Kyle|first1=Robert A.|last2=Larson|first2=Dirk R.|last3=McPhail|first3=Ellen D.|last4=Therneau|first4=Terry M.|last5=Dispenzieri|first5=Angela|last6=Kumar|first6=Shaji|last7=Kapoor|first7=Prashant|last8=Cerhan|first8=James R.|last9=Rajkumar|first9=S. Vincent|title=Fifty-Year Incidence of Waldenström Macroglobulinemia in Olmsted County, Minnesota, From 1961 Through 2010: A Population-Based Study With Complete Case Capture and Hematopathologic Review|journal=Mayo Clinic Proceedings|volume=93|issue=6|year=2018|pages=739–746|issn=00256196|doi=10.1016/j.mayocp.2018.02.011}}</ref>
*After 2000, a 2-fold increased mortality is reported in patients diagnosed with WM when compared with expected population [[mortality]].<ref name="KyleLarson2018">{{cite journal|last1=Kyle|first1=Robert A.|last2=Larson|first2=Dirk R.|last3=McPhail|first3=Ellen D.|last4=Therneau|first4=Terry M.|last5=Dispenzieri|first5=Angela|last6=Kumar|first6=Shaji|last7=Kapoor|first7=Prashant|last8=Cerhan|first8=James R.|last9=Rajkumar|first9=S. Vincent|title=Fifty-Year Incidence of Waldenström Macroglobulinemia in Olmsted County, Minnesota, From 1961 Through 2010: A Population-Based Study With Complete Case Capture and Hematopathologic Review|journal=Mayo Clinic Proceedings|volume=93|issue=6|year=2018|pages=739–746|issn=00256196|doi=10.1016/j.mayocp.2018.02.011}}</ref>
*The presence of symptoms is associated with a particularly poor prognosis among patients with the disease.
*The presence of symptoms is associated with a particularly poor prognosis among patients with the disease.
*Prognosis of [[asymptomatic]] patients is similar to that of the general population.<ref name="pmid15756000">{{cite journal| author=Gobbi PG, Baldini L, Broglia C, Goldaniga M, Comelli M, Morel P et al.| title=Prognostic validation of the international classification of immunoglobulin M gammopathies: a survival advantage for patients with immunoglobulin M monoclonal gammopathy of undetermined significance? | journal=Clin Cancer Res | year= 2005 | volume= 11 | issue= 5 | pages= 1786-90 | pmid=15756000 | doi=10.1158/1078-0432.CCR-04-1899 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15756000  }} </ref>
*Prognosis of [[asymptomatic]] patients is similar to that of the general population.<ref name="pmid15756000">{{cite journal| author=Gobbi PG, Baldini L, Broglia C, Goldaniga M, Comelli M, Morel P et al.| title=Prognostic validation of the international classification of immunoglobulin M gammopathies: a survival advantage for patients with immunoglobulin M monoclonal gammopathy of undetermined significance? | journal=Clin Cancer Res | year= 2005 | volume= 11 | issue= 5 | pages= 1786-90 | pmid=15756000 | doi=10.1158/1078-0432.CCR-04-1899 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15756000  }} </ref>


===Adverse prognostic factors===
===Adverse prognostic factors===
*Some of the pretreatment factors associated with shorter survival in WM patients are:<ref name="pmid12881396">{{cite journal| author=Dimopoulos MA, Hamilos G, Zervas K, Symeonidis A, Kouvatseas G, Roussou P et al.| title=Survival and prognostic factors after initiation of treatment in Waldenstrom's macroglobulinemia. | journal=Ann Oncol | year= 2003 | volume= 14 | issue= 8 | pages= 1299-305 | pmid=12881396 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881396  }} </ref><ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
*Some of the pre-treatment factors associated with shorter survival in WM patients are:<ref name="pmid12881396">{{cite journal| author=Dimopoulos MA, Hamilos G, Zervas K, Symeonidis A, Kouvatseas G, Roussou P et al.| title=Survival and prognostic factors after initiation of treatment in Waldenstrom's macroglobulinemia. | journal=Ann Oncol | year= 2003 | volume= 14 | issue= 8 | pages= 1299-305 | pmid=12881396 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12881396  }} </ref><ref name="pmid16611306">{{cite journal| author=Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM et al.| title=Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia. | journal=Br J Haematol | year= 2006 | volume= 133 | issue= 2 | pages= 158-64 | pmid=16611306 | doi=10.1111/j.1365-2141.2006.06003.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16611306  }} </ref>
**Age >/=65 years.
**Age >/=65 years.
**Organomegaly (Hepatosplenomegaly).
**[[Organomegaly]] ([[Hepatosplenomegaly]])
**B-symptoms (weight loss, fever or night sweats).
**B-symptoms (weight loss, fever or night sweats)
**Anaemia (Hb < 10.0 g/dl).
**[[Anemia]] (Hb < 10.0 g/dl)
**Platelets <100 x 10(6)/dl.
**[[Platelets]] <100 x 10(6)/dl
**Albumin <3.5 g/dl.
**[[Albumin]] <3.5 g/dl
**Bone marrow lymphoplasmacytic infiltrate >/=50%.
**Bone marrow lymphoplasmacytic infiltrate >/=50%.
**Elevated beta2-microglobulin ( associated with 3-fold increase in death).
**Elevated beta2-microglobulin ( associated with 3-fold increase in death)
**Leucopenia (<4.0 x 10(9)/l).
**[[Leukopenia]] (<4.0 x 10(9)/l)
**Thrombocytopenia (<150 x 10(9)/l).
**[[Thrombocytopenia]] (<150 x 10(9)/l)
**Quantitative IgM < 0.4 g/l.
**[[IgM|Quantitative IgM]] < 0.4 g/l
**Hyperviscosity.
**[[Hyperviscosity]]
*Other prognostic factors recently studied are:
*Other prognostic factors recently studied are:
**Serum free light chain.<ref name="pmid18452095">{{cite journal| author=Leleu X, Moreau AS, Weller E, Roccaro AM, Coiteux V, Manning R et al.| title=Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia. | journal=Leuk Lymphoma | year= 2008 | volume= 49 | issue= 6 | pages= 1104-7 | pmid=18452095 | doi=10.1080/10428190802074619 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18452095  }} </ref>
**Serum free light chain<ref name="pmid18452095">{{cite journal| author=Leleu X, Moreau AS, Weller E, Roccaro AM, Coiteux V, Manning R et al.| title=Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia. | journal=Leuk Lymphoma | year= 2008 | volume= 49 | issue= 6 | pages= 1104-7 | pmid=18452095 | doi=10.1080/10428190802074619 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18452095  }} </ref>
**Serum lactate dehydrogenase.<ref name="pmid19362972">{{cite journal| author=Kastritis E, Zervas K, Repoussis P, Michali E, Katodrytou E, Zomas A et al.| title=Prognostication in young and old patients with Waldenström's macroglobulinemia: importance of the International Prognostic Scoring System and of serum lactate dehydrogenase. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 1 | pages= 50-2 | pmid=19362972 | doi=10.3816/CLM.2009.n.012 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19362972  }} </ref>
**Serum [[lactate dehydrogenase]]<ref name="pmid19362972">{{cite journal| author=Kastritis E, Zervas K, Repoussis P, Michali E, Katodrytou E, Zomas A et al.| title=Prognostication in young and old patients with Waldenström's macroglobulinemia: importance of the International Prognostic Scoring System and of serum lactate dehydrogenase. | journal=Clin Lymphoma Myeloma | year= 2009 | volume= 9 | issue= 1 | pages= 50-2 | pmid=19362972 | doi=10.3816/CLM.2009.n.012 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19362972  }} </ref>
**Serum soluble CD27.<ref name="pmid18216294">{{cite journal| author=Ho AW, Hatjiharissi E, Ciccarelli BT, Branagan AR, Hunter ZR, Leleu X et al.| title=CD27-CD70 interactions in the pathogenesis of Waldenstrom macroglobulinemia. | journal=Blood | year= 2008 | volume= 112 | issue= 12 | pages= 4683-9 | pmid=18216294 | doi=10.1182/blood-2007-04-084525 | pmc=2597134 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18216294  }} </ref>
**Serum soluble [[CD27]]<ref name="pmid18216294">{{cite journal| author=Ho AW, Hatjiharissi E, Ciccarelli BT, Branagan AR, Hunter ZR, Leleu X et al.| title=CD27-CD70 interactions in the pathogenesis of Waldenstrom macroglobulinemia. | journal=Blood | year= 2008 | volume= 112 | issue= 12 | pages= 4683-9 | pmid=18216294 | doi=10.1182/blood-2007-04-084525 | pmc=2597134 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18216294  }} </ref>
*Most of the prognostic factors have defined the outcome of Waldenström macroglobulinemia in patients requiring treatment. However, very few studies have evaluated the prognostic factors in patients who don't initially need the treatment.
*Most of the prognostic factors have defined the outcome of Waldenström macroglobulinemia in patients requiring treatment. However, very few studies have evaluated the prognostic factors in patients who don't initially need the treatment.


Revision as of 17:52, 8 April 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2], Roukoz A. Karam, M.D.[3]; Grammar Reviewer: Natalie Harpenau, B.S.[4]

Overview

If left untreated, patients with asymptomatic Waldenström macroglobulinemia may progress to develop fatigue, weight loss, peripheral neuropathy and other symptoms of the disease. Common complications of Waldenström macroglobulinemia include: hyperviscosity syndrome, cold haemagglutinin disease, cryoglobulinemia, peripheral neuropathy, primary amyloidosis, renal insufficiency, malabsorbtive diarrhea, and visual abnormalities. Prognosis varies depending on the various factors involved. A five-year survival rate is 87% for low-risk disease and 36% for high-risk disease.

Natural History, Complications, and Prognosis

Natural History

Complications

Late and rare complications

Prognosis

  • Prognosis is generally poor.
  • The median survival from the time of diagnosis is 6.4 years.[12]
  • The median disease-specific survival is 11.2 years.[12]
  • Approximately 10% patients still live at 15 years.[13][14]
  • 5-year survival rate is 78%.
  • In the last decade (2001-2010), the median overall survival for all WM groups has improved to just over 8 years compared to 6 years in the previous decade (1991-2000).
  • After 2000, a 2-fold increased mortality is reported in patients diagnosed with WM when compared with expected population mortality.[15]
  • The presence of symptoms is associated with a particularly poor prognosis among patients with the disease.
  • Prognosis of asymptomatic patients is similar to that of the general population.[16]

Adverse prognostic factors

  • Some of the pre-treatment factors associated with shorter survival in WM patients are:[17][12]
  • Other prognostic factors recently studied are:
  • Most of the prognostic factors have defined the outcome of Waldenström macroglobulinemia in patients requiring treatment. However, very few studies have evaluated the prognostic factors in patients who don't initially need the treatment.

Risk Stratification Criteria

All the above prognostic data has been combined to risk stratify the WM patients and to formulate a standardized scoring system known as the International Prognostic Staging System for Waldenström's Macroglobulinemia (IPSSWM):[21]

Risk factors Score
Age > 65 1
Hemoglobin ≤ 11.5g/dl 1
Platelet ≤ 100,000μl 1
β-microglobulin > 3mg/l 1
IgM > 70g/l 1
International prognostic scoring system for Waldenström macroglobulinemia
Risk group Score 5-year survival
Low 0-1 (except age) 87%
Intermediate 2 or age>65 68%
High ≥3 36%
International prognostic scoring system for Waldenström macroglobulinemia
Risk group Score Median survival
Low 0-1 (except age) 12 years
Intermediate 2 or age>65 8 years
High ≥3 3.5 years

References

  1. ↑ Wang H, Chen Y, Li F, Delasalle K, Wang J, Alexanian R; et al. (2012). "Temporal and geographic variations of Waldenstrom macroglobulinemia incidence: a large population-based study". Cancer. 118 (15): 3793–800. doi:10.1002/cncr.26627. PMID 22139816.
  2. ↑ 2.0 2.1 García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A; et al. (2001). "Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases". Br J Haematol. 115 (3): 575–82. PMID 11736938.
  3. ↑ Michael AB, Lawes M, Kamalarajan M, Huissoon A, Pratt G (2004). "Cryoglobulinaemia as an acute presentation of Waldenstrom's macroglobulinaemia". Br J Haematol. 124 (5): 565. PMID 14871241.
  4. ↑ Levine T, Pestronk A, Florence J, Al-Lozi MT, Lopate G, Miller T; et al. (2006). "Peripheral neuropathies in Waldenström's macroglobulinaemia". J Neurol Neurosurg Psychiatry. 77 (2): 224–8. doi:10.1136/jnnp.2005.071175. PMC 2077569. PMID 16421127.
  5. ↑ 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.
  6. ↑ Owen RG, Pratt G, Auer RL, Flatley R, Kyriakou C, Lunn MP; et al. (2014). "Guidelines on the diagnosis and management of Waldenström macroglobulinaemia". Br J Haematol. 165 (3): 316–33. doi:10.1111/bjh.12760. PMID 24528152.
  7. ↑ Veloso FT, Fraga J, Saleiro JV (1988). "Macroglobulinemia and small intestinal disease. A case report with review of the literature". J Clin Gastroenterol. 10 (5): 546–50. PMID 3141496.
  8. ↑ Vos JM, Gustine J, Rennke HG, Hunter Z, Manning RJ, Dubeau TE; et al. (2016). "Renal disease related to Waldenström macroglobulinaemia: incidence, pathology and clinical outcomes". Br J Haematol. 175 (4): 623–630. doi:10.1111/bjh.14279. PMID 27468978.
  9. ↑ Civit T, Coulbois S, Baylac F, Taillandier L, Auque J (1997). "[Waldenström's macroglobulinemia and cerebral lymphoplasmocytic proliferation: Bing and Neel syndrome. Apropos of a new case]". Neurochirurgie. 43 (4): 245–9. PMID 9686227.
  10. ↑ Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH (2009). "Bing-Neel Syndrome revisited". Clin Lymphoma Myeloma. 9 (1): 104–6. doi:10.3816/CLM.2009.n.028. PMID 19362988.
  11. ↑ Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG (2009). "Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options". Clin Lymphoma Myeloma. 9 (6): 462–6. doi:10.3816/CLM.2009.n.091. PMID 19951888.
  12. ↑ 12.0 12.1 12.2 Ghobrial IM, Fonseca R, Gertz MA, Plevak MF, Larson DR, Therneau TM; et al. (2006). "Prognostic model for disease-specific and overall mortality in newly diagnosed symptomatic patients with Waldenstrom macroglobulinaemia". Br J Haematol. 133 (2): 158–64. doi:10.1111/j.1365-2141.2006.06003.x. PMID 16611306.
  13. ↑ Morel, P.; Duhamel, A.; Gobbi, P.; Dimopoulos, M. A.; Dhodapkar, M. V.; McCoy, J.; Crowley, J.; Ocio, E. M.; Garcia-Sanz, R.; Treon, S. P.; Leblond, V.; Kyle, R. A.; Barlogie, B.; Merlini, G. (2009). "International prognostic scoring system for Waldenstrom macroglobulinemia". Blood. 113 (18): 4163–4170. doi:10.1182/blood-2008-08-174961. ISSN 0006-4971.
  14. ↑ Kyle RA, Greipp PR, Gertz MA, Witzig TE, Lust JA, Lacy MQ; et al. (2000). "Waldenström's macroglobulinaemia: a prospective study comparing daily with intermittent oral chlorambucil". Br J Haematol. 108 (4): 737–42. PMID 10792277.
  15. ↑ Kyle, Robert A.; Larson, Dirk R.; McPhail, Ellen D.; Therneau, Terry M.; Dispenzieri, Angela; Kumar, Shaji; Kapoor, Prashant; Cerhan, James R.; Rajkumar, S. Vincent (2018). "Fifty-Year Incidence of Waldenström Macroglobulinemia in Olmsted County, Minnesota, From 1961 Through 2010: A Population-Based Study With Complete Case Capture and Hematopathologic Review". Mayo Clinic Proceedings. 93 (6): 739–746. doi:10.1016/j.mayocp.2018.02.011. ISSN 0025-6196.
  16. ↑ Gobbi PG, Baldini L, Broglia C, Goldaniga M, Comelli M, Morel P; et al. (2005). "Prognostic validation of the international classification of immunoglobulin M gammopathies: a survival advantage for patients with immunoglobulin M monoclonal gammopathy of undetermined significance?". Clin Cancer Res. 11 (5): 1786–90. doi:10.1158/1078-0432.CCR-04-1899. PMID 15756000.
  17. ↑ Dimopoulos MA, Hamilos G, Zervas K, Symeonidis A, Kouvatseas G, Roussou P; et al. (2003). "Survival and prognostic factors after initiation of treatment in Waldenstrom's macroglobulinemia". Ann Oncol. 14 (8): 1299–305. PMID 12881396.
  18. ↑ Leleu X, Moreau AS, Weller E, Roccaro AM, Coiteux V, Manning R; et al. (2008). "Serum immunoglobulin free light chain correlates with tumor burden markers in Waldenstrom macroglobulinemia". Leuk Lymphoma. 49 (6): 1104–7. doi:10.1080/10428190802074619. PMID 18452095.
  19. ↑ Kastritis E, Zervas K, Repoussis P, Michali E, Katodrytou E, Zomas A; et al. (2009). "Prognostication in young and old patients with Waldenström's macroglobulinemia: importance of the International Prognostic Scoring System and of serum lactate dehydrogenase". Clin Lymphoma Myeloma. 9 (1): 50–2. doi:10.3816/CLM.2009.n.012. PMID 19362972.
  20. ↑ Ho AW, Hatjiharissi E, Ciccarelli BT, Branagan AR, Hunter ZR, Leleu X; et al. (2008). "CD27-CD70 interactions in the pathogenesis of Waldenstrom macroglobulinemia". Blood. 112 (12): 4683–9. doi:10.1182/blood-2007-04-084525. PMC 2597134. PMID 18216294.
  21. ↑ Morel P, Duhamel A, Gobbi P, Dimopoulos MA, Dhodapkar MV, McCoy J; et al. (2009). "International prognostic scoring system for Waldenstrom macroglobulinemia". Blood. 113 (18): 4163–70. doi:10.1182/blood-2008-08-174961. PMID 19196866.

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