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==Overview==
==Overview==
[[Risk stratification tools|Risk stratification]] determines the protocol of management used for lymphoplasmacytic lymphoma. There is no treatment for asymptomatic lymphoplasmacytic lymphoma. The mainstay of treatment for symptomatic lymphoplasmacytic lymphoma is [[Rituximab]] +/- [[Chemotherapy]]. [[Hyperviscosity syndrome]] is a medical emergency and requires prompt treatment with [[plasmapheresis]].
[[Risk stratification tools|Risk stratification]] determines the protocol of management used for [[lymphoplasmacytic lymphoma]]. There is no treatment for [[asymptomatic]] [[lymphoplasmacytic lymphoma]]. The mainstay of treatment for [[symptomatic]] [[lymphoplasmacytic lymphoma]] is [[Rituximab]] +/- [[Chemotherapy]]. [[Hyperviscosity syndrome]] is a [[medical emergency]] and requires prompt treatment with [[plasmapheresis]].


==Medical Therapy==
==Medical Therapy==
There's no cure for WM/LPL with current therapies. Instead, the treatment goals are to control symptoms and prevent end-organ damage, while maximizing quality of life. There is no standard therapy for the treatment of LPL. While various drugs and combinations have demonstrated to have provided clinical benefit, hence, there are several different options for treating lymphoplasmacytic lymphoma depending on stage of the disease:<ref name="Tx">Lymphoplasmacytic lymphoma. Canadian Cancer Society 2015. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/lymphoplasmacytic-lymphoma/?region=ab Accessed on November 6 2015 </ref>
There's no [[cure]] for WM/LPL with current therapies. Instead, the treatment goals are to control [[symptoms]] and prevent end-organ damage, while maximizing [[quality of life]]. There is no standard [[therapy]] for the treatment of LPL. While various [[drugs]] and combinations have demonstrated to have provided [[clinical]] benefit, hence, there are several different options for treating [[lymphoplasmacytic lymphoma]] depending on stage of the disease:<ref name="Tx">Lymphoplasmacytic lymphoma. Canadian Cancer Society 2015. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/lymphoplasmacytic-lymphoma/?region=ab Accessed on November 6 2015 </ref>


====Watchful waiting/active surveillance for asymptomatic/Smoldering Waldenström's Macroglobulinemia/LPL====
====Watchful waiting/active surveillance for asymptomatic patients with LPL:====
There is no treatment for asymptomatic Waldenström macroglobulinemia/LPL. As LPL develops slowly and may not need to be treated right away, it is monitored by healthcare team every 3-6 months which is known as watchful waiting/active surveillance and treatment is started when symptoms appear, such as hyperviscosity syndrome, or there are signs that the disease is progressing more quickly.<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref> Active surveillance includes monitoring of the following laboratory parameters:
There is no treatment for [[asymptomatic]] patients with LPL. As LPL develops slowly and may not need to be treated right away, it is monitored by [[Health care|healthcare]] team every 3-6 months which is known as [[watchful waiting]]/active surveillance and treatment is started when [[symptoms]] appear, such as [[hyperviscosity syndrome]], or there are [[signs]] that the [[disease]] is progressing more quickly.<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref> Active surveillance includes monitoring of the following laboratory parameters:
*Complete blood count ([[Complete blood count|CBC]]) with differential
*[[Complete blood count]] ([[Complete blood count|CBC]]) with differential.
*Complete metabolic panel ([[CMP-N-acetylneuraminate monooxygenase|CMP]])
*Complete metabolic panel ([[CMP-N-acetylneuraminate monooxygenase|CMP]]).
*Immunoglobulin levels in the serum (quantitative)
*[[Immunoglobulin]] levels in the [[serum]] (quantitative).
*Serum protein electrophoresis
*[[Serum protein electrophoresis]].


====Symptomatic Waldenström's Macroglobulinemia/LPL====
====Symptomatic patients with LPL:====
Symptomatic patients with waldenström macroglobulinemia/LPL are started on chemotherapy depending on the stage.<ref name="ADR">Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015</ref>
[[Symptomatic]] patients with LPL are started on [[chemotherapy]] depending on the stage.<ref name="ADR">Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015</ref>


*Initial stage of waldenström's macroglobulinemia/LPL associated with:
*Initial stage of LPL is associated with:
:*[[Neuropathy]]
:*[[Neuropathy]].
:*[[Anemia]] or [[cytopenias]]
:*[[Anemia]] or [[cytopenias]].
:*Low-volume nodal involvement  
:*Low-volume nodal involvement.
:*Asymptomatic [[splenomegaly]]
:*[[Asymptomatic]] [[splenomegaly]].


*Late stage of Waldenström's macroglobulinemia/LPL associated with:
*Late stage of LPL is associated with:
:*[[Adenopathy]]
:*[[Adenopathy]].
:*Symptomatic [[splenomegaly]]  
:*[[Symptomatic]] [[splenomegaly]].
:*[[Cytopenia|Cytopenias]]
:*[[Cytopenia|Cytopenias]].
:*[[Hyperviscosity syndrome]]
:*[[Hyperviscosity syndrome]].
:*[[Neuropathy]]
:*[[Neuropathy]].
:*Constitutional symptoms
:*Constitutional [[symptoms]].
*Men and women with childbearing potential should receive counseling about the potential effect of treatment on their fertility and options for fertility-preserving measures.
*[[Men]] and women with childbearing potential should receive [[counseling]] about the potential effect of treatment on their [[fertility]] and options for [[fertility]]-preserving measures.
*Chemotherapy drugs that may be used with or without prednisone include:  
*[[Chemotherapy]] [[drugs]] that may be used with or without [[prednisone]] include:  
**Chlorambucil (Leukeran)
**[[Chlorambucil]] ([[Leukeran]]).
**Fludarabine (Fludara)
**[[Fludarabine]] ([[Fludara]]).
**Bendamustine (Treanda)
**[[Bendamustine]] ([[Treanda]]).
**Cyclophosphamide (Cytoxan, Procytox)
**[[Cyclophosphamide]] ([[Cytoxan]], Procytox).
 
*Combinations of chemotherapy drugs that may be used include:
**DRC – dexamethasone (Decadron, Dexasone), rituximab (Rituxan) and cyclophosphamide
**BRD – bortezomib (Velcade) and rituximab, with or without dexamethasone
**CVP – cyclophosphamide, vincristine (Oncovin) and prednisone
**R-CVP – CVP with rituximab
**Thalidomide (Thalomid) and rituximab


*Combinations of [[chemotherapy]] [[drugs]] that may be used include:
**DRC – [[dexamethasone]] ([[Decadron]], [[Dexasone]]), [[rituximab]] ([[Rituxan]]) and [[cyclophosphamide]].
**BRD – [[bortezomib]] ([[Velcade]]) and [[rituximab]], with or without [[dexamethasone]].
**CVP – [[cyclophosphamide]], [[vincristine]] (Oncovin) and [[prednisone]].
**R-CVP – CVP with [[rituximab]].
**[[Thalidomide]] ([[Thalomid]]) and [[rituximab]].


{| style="border: 0px; font-size: 90%; margin: 3px; width: 800px"
{| style="border: 0px; font-size: 90%; margin: 3px; width: 800px"
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*[[Rituximab]]
*[[Rituximab]]
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*Infusion related reaction
*[[Infusion-related reaction|Infusion related reaction]]
*[[Hepatitis B]] reaction
*[[Hepatitis B]] reaction
*Progressive multi-focal leukoencephaloptahy
*Progressive multi-focal leukoencephaloptahy
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| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Anemia]]
*[[Anemia]]
*Neurological symptoms
*[[Neurological]] [[symptoms]]
*Symptomatic [[cryoglobulinemia]]
*[[Symptomatic]] [[cryoglobulinemia]]
*[[Thrombocytopenia]]
*[[Thrombocytopenia]]
|-
|-
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| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Anemia]]
*[[Anemia]]
*Neurological symptoms
*[[Neurological]] [[symptoms]]
*Symptomatic [[cryoglobulinemia]]
*[[Symptomatic]] [[cryoglobulinemia]]
*[[Thrombocytopenia]]
*[[Thrombocytopenia]]
*[[Atrial fibrillation]]
*[[Atrial fibrillation]]
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|}
|}


====Hyperviscosity syndrome====
====Hyperviscosity syndrome:====
*Lymphoplasmacytic lymphoma complicated with [[hyperviscosity syndrome]] is a medical emergency and requires prompt treatment with plasmapheresis.<ref name="ADR">Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015</ref>
*[[Lymphoplasmacytic lymphoma]] complicated with [[hyperviscosity syndrome]] is a [[medical emergency]] and requires prompt treatment with [[plasmapheresis]].<ref name="ADR">Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015</ref>
*[[Plasmapheresis]] temporarily lowers [[IgM]] levels by removing some of the abnormal IgM from the blood, which makes blood thinner.  
*[[Plasmapheresis]] temporarily lowers [[IgM]] levels by removing some of the abnormal [[IgM]] from the [[blood]], which makes [[blood]] thinner.  
*Plasmapheresis is usually given until chemotherapy starts to work.
*[[Plasmapheresis]] is usually given until [[chemotherapy]] starts to work.
*Plasmapheresis is combined with chemotherapy to control the disease for a longer period of time.
*[[Plasmapheresis]] is combined with [[chemotherapy]] to control the [[disease]] for a longer period of time.
*Plasmapheresis is also used in WM patients with [[hemolysis]].
*[[Plasmapheresis]] is also used in WM patients with [[hemolysis]].


===Initial treatment of Lymphoplasmacytic lymphoma===
===Initial treatment of Lymphoplasmacytic lymphoma:===
{{familytree/start}}
{{familytree/start}}
{{familytree | | | | | | | | A01 |A01=Does the patient has an indication for LPL/WM treatment? B symptoms (recurrent fever, night sweats, weight loss, fatigue, hyperviscosity, bulky/symptomatic lymphadenopathy, symptomatic hepatosplenomegaly, symptomatic organomegaly or organ/tissue infiltration, WM associated peripheral neuropathy, cold agglutinin hemolytic anemia, symptomatic cryoglobulinemia, immune hemolytic anemia and/or thrombocytopenia, WM associated AL amyloidosis, WM associated nephropathy, hemoglobin = or < 10g/dl, platelet count = or < 100 x 10'9/L.}}
{{familytree | | | | | | | | A01 |A01=Does the patient has an indication for LPL/WM treatment? B symptoms (recurrent fever, night sweats, weight loss, fatigue, hyperviscosity, bulky/symptomatic lymphadenopathy, symptomatic hepatosplenomegaly, symptomatic organomegaly or organ/tissue infiltration, WM associated peripheral neuropathy, cold agglutinin hemolytic anemia, symptomatic cryoglobulinemia, immune hemolytic anemia and/or thrombocytopenia, WM associated AL amyloidosis, WM associated nephropathy, hemoglobin = or < 10g/dl, platelet count = or < 100 x 10'9/L.}}
Line 176: Line 175:
{{familytree/end}}
{{familytree/end}}


===Drug of choice for Bing-Neel Syndrome===
===Drug of choice for Bing-Neel Syndrome:===
Many recent studies have shown to be Ibrutinib (560mg), an oral Bruton's tyrosine kinase inhibitor, with or without concurrent Rituximab, as a drug of choice for treatment of Bing-Neel syndrome. It works by penetrating the blood brain barrier.<ref name="pmid30228918">{{cite journal| author=O'Neil DS, Francescone MA, Khan K, Bachir A, O'Connor OA, Sawas A| title=A Case of Bing-Neel Syndrome Successfully Treated with Ibrutinib. | journal=Case Rep Hematol | year= 2018 | volume= 2018 | issue=  | pages= 8573105 | pmid=30228918 | doi=10.1155/2018/8573105 | pmc=6136466 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30228918  }} </ref><ref name="pmid27758817">{{cite journal| author=Minnema MC, Kimby E, D'Sa S, Fornecker LM, Poulain S, Snijders TJ et al.| title=Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome. | journal=Haematologica | year= 2017 | volume= 102 | issue= 1 | pages= 43-51 | pmid=27758817 | doi=10.3324/haematol.2016.147728 | pmc=5210231 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27758817  }} </ref><ref name="pmid30279255">{{cite journal| author=Tallant A, Selig D, Wanko SO, Roswarski J| title=First-line ibrutinib for Bing-Neel syndrome. | journal=BMJ Case Rep | year= 2018 | volume= 2018 | issue=  | pages=  | pmid=30279255 | doi=10.1136/bcr-2018-226102 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30279255  }} </ref><ref name="pmid26689870">{{cite journal| author=Cabannes-Hamy A, Lemal R, Goldwirt L, Poulain S, Amorim S, Pérignon R et al.| title=Efficacy of ibrutinib in the treatment of Bing-Neel syndrome. | journal=Am J Hematol | year= 2016 | volume= 91 | issue= 3 | pages= E17-9 | pmid=26689870 | doi=10.1002/ajh.24279 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26689870  }} </ref><ref name="pmid27409073">{{cite journal| author=Mason C, Savona S, Rini JN, Castillo JJ, Xu L, Hunter ZR et al.| title=Ibrutinib penetrates the blood brain barrier and shows efficacy in the therapy of Bing Neel syndrome. | journal=Br J Haematol | year= 2017 | volume= 179 | issue= 2 | pages= 339-341 | pmid=27409073 | doi=10.1111/bjh.14218 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27409073  }} </ref>
Many recent studies have shown to be Ibrutinib (560mg), an oral Bruton's tyrosine kinase inhibitor, with or without concurrent Rituximab, as a drug of choice for treatment of Bing-Neel syndrome. It works by penetrating the blood brain barrier.<ref name="pmid30228918">{{cite journal| author=O'Neil DS, Francescone MA, Khan K, Bachir A, O'Connor OA, Sawas A| title=A Case of Bing-Neel Syndrome Successfully Treated with Ibrutinib. | journal=Case Rep Hematol | year= 2018 | volume= 2018 | issue=  | pages= 8573105 | pmid=30228918 | doi=10.1155/2018/8573105 | pmc=6136466 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30228918  }} </ref><ref name="pmid27758817">{{cite journal| author=Minnema MC, Kimby E, D'Sa S, Fornecker LM, Poulain S, Snijders TJ et al.| title=Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome. | journal=Haematologica | year= 2017 | volume= 102 | issue= 1 | pages= 43-51 | pmid=27758817 | doi=10.3324/haematol.2016.147728 | pmc=5210231 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27758817  }} </ref><ref name="pmid30279255">{{cite journal| author=Tallant A, Selig D, Wanko SO, Roswarski J| title=First-line ibrutinib for Bing-Neel syndrome. | journal=BMJ Case Rep | year= 2018 | volume= 2018 | issue=  | pages=  | pmid=30279255 | doi=10.1136/bcr-2018-226102 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30279255  }} </ref><ref name="pmid26689870">{{cite journal| author=Cabannes-Hamy A, Lemal R, Goldwirt L, Poulain S, Amorim S, Pérignon R et al.| title=Efficacy of ibrutinib in the treatment of Bing-Neel syndrome. | journal=Am J Hematol | year= 2016 | volume= 91 | issue= 3 | pages= E17-9 | pmid=26689870 | doi=10.1002/ajh.24279 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26689870  }} </ref><ref name="pmid27409073">{{cite journal| author=Mason C, Savona S, Rini JN, Castillo JJ, Xu L, Hunter ZR et al.| title=Ibrutinib penetrates the blood brain barrier and shows efficacy in the therapy of Bing Neel syndrome. | journal=Br J Haematol | year= 2017 | volume= 179 | issue= 2 | pages= 339-341 | pmid=27409073 | doi=10.1111/bjh.14218 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27409073  }} </ref>


One or more of the following treatments can be given for lymphoplasmacytic lymphoma.
One or more of the following treatments can be given for lymphoplasmacytic lymphoma.
=====Targeted therapy=====
=====Targeted therapy:=====
*Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.  
*Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.  
*Targeted therapy drugs used alone or in combination to treat lymphoplasmacytic lymphoma include rituximab, bortezomib and ibrutinib (Imbruvica).
*Targeted therapy drugs used alone or in combination to treat lymphoplasmacytic lymphoma include rituximab, bortezomib and ibrutinib (Imbruvica).


=====Immunotherapy=====
=====Immunotherapy:=====
*Immunotherapy works by stimulating, boosting, restoring or acting like the body’s immune system to create a response against cancer cells. Immunomodulatory drugs are a type of immunotherapy that interferes with the growth and division of cancer cells.
*Immunotherapy works by stimulating, boosting, restoring or acting like the body’s immune system to create a response against cancer cells. Immunomodulatory drugs are a type of immunotherapy that interferes with the growth and division of cancer cells.
*Thalidomide is a type of immunomodulatory drug that may be used to treat lymphoplasmacytic lymphoma.
*Thalidomide is a type of immunomodulatory drug that may be used to treat lymphoplasmacytic lymphoma.


=====Radiation therapy=====
=====Radiation therapy:=====
In some rare cases, external beam radiation therapy may be required to treat LPL that develops outside of the lymphatic system (called extralymphatic disease).
In some rare cases, external beam radiation therapy may be required to treat LPL that develops outside of the lymphatic system (called extralymphatic disease).


Revision as of 23:18, 19 February 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]

Overview

Risk stratification determines the protocol of management used for lymphoplasmacytic lymphoma. There is no treatment for asymptomatic lymphoplasmacytic lymphoma. The mainstay of treatment for symptomatic lymphoplasmacytic lymphoma is Rituximab +/- Chemotherapy. Hyperviscosity syndrome is a medical emergency and requires prompt treatment with plasmapheresis.

Medical Therapy

There's no cure for WM/LPL with current therapies. Instead, the treatment goals are to control symptoms and prevent end-organ damage, while maximizing quality of life. There is no standard therapy for the treatment of LPL. While various drugs and combinations have demonstrated to have provided clinical benefit, hence, there are several different options for treating lymphoplasmacytic lymphoma depending on stage of the disease:[1]

Watchful waiting/active surveillance for asymptomatic patients with LPL:

There is no treatment for asymptomatic patients with LPL. As LPL develops slowly and may not need to be treated right away, it is monitored by healthcare team every 3-6 months which is known as watchful waiting/active surveillance and treatment is started when symptoms appear, such as hyperviscosity syndrome, or there are signs that the disease is progressing more quickly.[2] Active surveillance includes monitoring of the following laboratory parameters:

Symptomatic patients with LPL:

Symptomatic patients with LPL are started on chemotherapy depending on the stage.[3]

  • Initial stage of LPL is associated with:
  • Late stage of LPL is associated with:
Treatment Regimen[3]

Drugs Side effects

CHOP-R regimen

Ibrutinib

Rituximab

FR regimen

BDR regimen

DRC regimen

CR regimen

IR regimen

Helical computed tomographic scanning showed ground-glass shadowing in bilateral lungs before prednisone treatment and a recovery at 1 week post-treatment.Source: Bai X. et al, Department of Hematology, Beijing Tiantan Hospital, Capital Medical University 6 Tiantan Xili Dongcheng District, Beijing, 100050, China.

Hyperviscosity syndrome:

Initial treatment of Lymphoplasmacytic lymphoma:

 
 
 
 
 
 
 
Does the patient has an indication for LPL/WM treatment? B symptoms (recurrent fever, night sweats, weight loss, fatigue, hyperviscosity, bulky/symptomatic lymphadenopathy, symptomatic hepatosplenomegaly, symptomatic organomegaly or organ/tissue infiltration, WM associated peripheral neuropathy, cold agglutinin hemolytic anemia, symptomatic cryoglobulinemia, immune hemolytic anemia and/or thrombocytopenia, WM associated AL amyloidosis, WM associated nephropathy, hemoglobin = or < 10g/dl, platelet count = or < 100 x 10'9/L.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes
 
 
 
 
 
 
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Does the patient has symptoms associated with hyperviscosity such as: Oronasal bleeding, blurred vision, headaches, dizziness, paresthesias, retinal vein engorgement, flame-shaped hemorrhages, papilledema, stupor or coma.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No
 
Yes
 
 
 
 
 
For smoldering/asymptomatic WM/LPL, just follow up every 4-6 months with CBC and monoclonal protein levels.
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Assess degree of symptom burden in WM/LPL pateint.
 
Consider emergent plasmapheresis for treatment of hyperviscosity
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Low
 
Moderate/High
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Following are the 2 options for patients with low tumor burden with minimal symptoms:
  • Single agent Rituximab.
  • Rituximab + chemotherapy as with high burden disease.
 
Following 2 are the preferred regimens for moderate/severe symptoms or high tumor burden:
  • Bendamustine + rituximab.
  • Dexamethasone & rituximab + cyclophosphamide
    •  
     
     
     
     
     

    Drug of choice for Bing-Neel Syndrome:

    Many recent studies have shown to be Ibrutinib (560mg), an oral Bruton's tyrosine kinase inhibitor, with or without concurrent Rituximab, as a drug of choice for treatment of Bing-Neel syndrome. It works by penetrating the blood brain barrier.[4][5][6][7][8]

    One or more of the following treatments can be given for lymphoplasmacytic lymphoma.

    Targeted therapy:
    • Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.
    • Targeted therapy drugs used alone or in combination to treat lymphoplasmacytic lymphoma include rituximab, bortezomib and ibrutinib (Imbruvica).
    Immunotherapy:
    • Immunotherapy works by stimulating, boosting, restoring or acting like the body’s immune system to create a response against cancer cells. Immunomodulatory drugs are a type of immunotherapy that interferes with the growth and division of cancer cells.
    • Thalidomide is a type of immunomodulatory drug that may be used to treat lymphoplasmacytic lymphoma.
    Radiation therapy:

    In some rare cases, external beam radiation therapy may be required to treat LPL that develops outside of the lymphatic system (called extralymphatic disease).

    References

    1. ↑ Lymphoplasmacytic lymphoma. Canadian Cancer Society 2015. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/lymphoplasmacytic-lymphoma/?region=ab Accessed on November 6 2015
    2. ↑ Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015
    3. ↑ 3.0 3.1 3.2 Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015
    4. ↑ O'Neil DS, Francescone MA, Khan K, Bachir A, O'Connor OA, Sawas A (2018). "A Case of Bing-Neel Syndrome Successfully Treated with Ibrutinib". Case Rep Hematol. 2018: 8573105. doi:10.1155/2018/8573105. PMC 6136466. PMID 30228918.
    5. ↑ Minnema MC, Kimby E, D'Sa S, Fornecker LM, Poulain S, Snijders TJ; et al. (2017). "Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome". Haematologica. 102 (1): 43–51. doi:10.3324/haematol.2016.147728. PMC 5210231. PMID 27758817.
    6. ↑ Tallant A, Selig D, Wanko SO, Roswarski J (2018). "First-line ibrutinib for Bing-Neel syndrome". BMJ Case Rep. 2018. doi:10.1136/bcr-2018-226102. PMID 30279255.
    7. ↑ Cabannes-Hamy A, Lemal R, Goldwirt L, Poulain S, Amorim S, Pérignon R; et al. (2016). "Efficacy of ibrutinib in the treatment of Bing-Neel syndrome". Am J Hematol. 91 (3): E17–9. doi:10.1002/ajh.24279. PMID 26689870.
    8. ↑ Mason C, Savona S, Rini JN, Castillo JJ, Xu L, Hunter ZR; et al. (2017). "Ibrutinib penetrates the blood brain barrier and shows efficacy in the therapy of Bing Neel syndrome". Br J Haematol. 179 (2): 339–341. doi:10.1111/bjh.14218. PMID 27409073.

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