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{{Waldenström's macroglobulinemia}}
{{Waldenström's macroglobulinemia}}
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==Overview==
==Overview==
==Medical Therapy==
==Medical Therapy==
There are several different options for Waldenström Macroglobulinemia.<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 are several different options for Waldenström Macroglobulinemia.<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><ref name=TXP>Waldenstrom Macroglobulinemia Treatment Protocols. Medscape 2015. http://emedicine.medscape.com/article/2057687-overview Accessed on November 6 2015 </ref>


===Watchful Waiting===
==Asymptomatic/Smoldering Waldenström's macroglobulinemia==
Patients who do not have symptoms and whose cancer does not seem to be progressing often do not need treatment.
Patients who do not have symptoms and whose cancer does not seem to be progressing often do not need treatment.
They can be monitored every 3-6 months - a 'wait and watch approach'.<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref>
They can be monitored every 3-6 months - a 'wait and watch approach'.<ref name="BM">Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015</ref>


===Plasmapheresis===
==Symptomatic Waldenström's macroglobulinemia==
*Plasmapheresis is recommended in patients with Waldenström Macroglobulinemia who develop hyperviscosity symptoms.  
Symptomatic patients with Waldenström Macroglobulinemia are started on chemotherapy depending on the stage.
 
===Initial stage of Waldenström's macroglobulinemia associated with===
*Neuropathy,
*Anemia or cytopenias,
*Low-volume nodal involvement, and
*Asymptomatic splenomegaly
'''Treatment:''' Single-agent Rituximab therapy
 
===Late stage of Waldenström's macroglobulinemia associated with===
*Adenopathy,
*Symptomatic splenomegaly,
*Cytopenias,
*Hyperviscosity syndrome,
*Neuropathy, or
*Constitutional symptoms
 
'''Treatment regimen used:'''<ref name="PHARM">Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/2057687-overview Accessed on November 11, 2015</ref>
 
'''CHOP-R regimen:'''
*Cyclophosphamide 750 mg/m2 IV day 1;
*Doxorubicin 50 mg/m2;
*Vincristine 1.4 mg/m2 IV (max 2 mg) day 1;
*Prednisone 50 mg/m2 PO days 1-5;
*Rituximab 375 mg/m2 IV day 1;
*Primary: Repeat 21 day cycle for 6-8 cycles
 
'''Ibrutinib''' 420 mg PO once daily until disease progression
 
'''Rituximab''' 375 mg/m2 IV once weekly x 4 weeks
 
'''Fludarabine/Rituximab regimen:'''
*Fludarabine 25 mg/m2 IV days 1-5;
*Rituximab 375 mg/m2 IV day 1;
*Repeat 28 day cycle for 4-6 cycles
 
'''FCR regimen:'''
*Fludarabine 25 mg/m2 IV days 1-3
*Cyclophosphamide 250 mg/m2 IV days 1-3
*Rituximab 375 mg/m2 IV day 1
*Primary: Repeat 28 day cycle for 4-6 cycles
*May also be given with mitoxantrone 10 mg/m2 on day 1
 
'''BR regimen:'''
*Bendamustine 90 mg/m2 IV days 1-2
*Rituximab 375 mg/m2 IV day 1
*Primary: Repeat 21 day cycle for 6 cycles
 
'''BDR regimen:'''
*Bortezomib 1.3 mg/m2plus
*Dexamethasone 40 mg IV days 1, 4, 8, and 11
*Rituximab 375 mg/m2 IV day 11
*Primary: Repeat 21 day cycle for 4 cycles
 
'''DRC regimen:'''
*Dexamethasone 20 mg IV day 1
*Rituximab 375 mg/m2 IV day 1
*Cyclophosphamide 100 mg/m2 PO BID days 1-5
*Primary: Repeat 21 day cycle for 6 cycles
 
'''CR regimen:'''
*Cladribine 0.1 mg/kg SC days 1-5
*Rituximab 375 mg/m2 IV day 1
*Primary: Repeat 28 day cycle for 4 cycles
 
===Hyperviscosity syndrome===
*Plasmapheresis is recommended emergent treatment option for patients with Waldenström Macroglobulinemia who develop hyperviscosity symptoms. <ref name="PHARM">Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/2057687-overview Accessed on November 11, 2015</ref>
*Plasmapheresis temporarily lowers IgM levels by removing some of the abnormal IgM from the blood, which makes the blood thinner.  
*Plasmapheresis temporarily lowers IgM levels by removing some of the abnormal IgM from the blood, which makes the blood thinner.  
*However, plasmapheresis does not affect the lymphoma cells.
*However, plasmapheresis does not affect the lymphoma cells.
Line 17: Line 83:
*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.


===Chemotherapy===
==Salvage approach==
*Patients with Waldenström Macroglobulinemia who symptomatic are started on chemotherapy.
*Stem cell transplant is used in patients whose lymphoma relapses or is not responding to other treatments (refractory). <ref name="PHARM">Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/2057687-overview Accessed on November 11, 2015</ref>
*Drgus:
**chlorambucil or cyclophosphamide, with or without prednisone
**fludarabine or cladribine
 
===Biological therapy===
*Rituximab may be used alone or in combination with chemotherapy.
 
===Stem cell transplant===
*Stem cell transplant is used in patients whose lymphoma relapses or is not responding to other treatments (refractory).
 
==References==
==References==
{{reflist|2}}
{{reflist|2}}

Revision as of 17:34, 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

Medical Therapy

There are several different options for Waldenström Macroglobulinemia.[1][2]

Asymptomatic/Smoldering Waldenström's macroglobulinemia

Patients who do not have symptoms and whose cancer does not seem to be progressing often do not need treatment. They can be monitored every 3-6 months - a 'wait and watch approach'.[3]

Symptomatic Waldenström's macroglobulinemia

Symptomatic patients with Waldenström Macroglobulinemia are started on chemotherapy depending on the stage.

Initial stage of Waldenström's macroglobulinemia associated with

  • Neuropathy,
  • Anemia or cytopenias,
  • Low-volume nodal involvement, and
  • Asymptomatic splenomegaly

Treatment: Single-agent Rituximab therapy

Late stage of Waldenström's macroglobulinemia associated with

  • Adenopathy,
  • Symptomatic splenomegaly,
  • Cytopenias,
  • Hyperviscosity syndrome,
  • Neuropathy, or
  • Constitutional symptoms

Treatment regimen used:[4]

CHOP-R regimen:

  • Cyclophosphamide 750 mg/m2 IV day 1;
  • Doxorubicin 50 mg/m2;
  • Vincristine 1.4 mg/m2 IV (max 2 mg) day 1;
  • Prednisone 50 mg/m2 PO days 1-5;
  • Rituximab 375 mg/m2 IV day 1;
  • Primary: Repeat 21 day cycle for 6-8 cycles

Ibrutinib 420 mg PO once daily until disease progression

Rituximab 375 mg/m2 IV once weekly x 4 weeks

Fludarabine/Rituximab regimen:

  • Fludarabine 25 mg/m2 IV days 1-5;
  • Rituximab 375 mg/m2 IV day 1;
  • Repeat 28 day cycle for 4-6 cycles

FCR regimen:

  • Fludarabine 25 mg/m2 IV days 1-3
  • Cyclophosphamide 250 mg/m2 IV days 1-3
  • Rituximab 375 mg/m2 IV day 1
  • Primary: Repeat 28 day cycle for 4-6 cycles
  • May also be given with mitoxantrone 10 mg/m2 on day 1

BR regimen:

  • Bendamustine 90 mg/m2 IV days 1-2
  • Rituximab 375 mg/m2 IV day 1
  • Primary: Repeat 21 day cycle for 6 cycles

BDR regimen:

  • Bortezomib 1.3 mg/m2plus
  • Dexamethasone 40 mg IV days 1, 4, 8, and 11
  • Rituximab 375 mg/m2 IV day 11
  • Primary: Repeat 21 day cycle for 4 cycles

DRC regimen:

  • Dexamethasone 20 mg IV day 1
  • Rituximab 375 mg/m2 IV day 1
  • Cyclophosphamide 100 mg/m2 PO BID days 1-5
  • Primary: Repeat 21 day cycle for 6 cycles

CR regimen:

  • Cladribine 0.1 mg/kg SC days 1-5
  • Rituximab 375 mg/m2 IV day 1
  • Primary: Repeat 28 day cycle for 4 cycles

Hyperviscosity syndrome

  • Plasmapheresis is recommended emergent treatment option for patients with Waldenström Macroglobulinemia who develop hyperviscosity symptoms. [4]
  • Plasmapheresis temporarily lowers IgM levels by removing some of the abnormal IgM from the blood, which makes the blood thinner.
  • However, plasmapheresis does not affect the lymphoma cells.
  • Plasmapheresis is usually given until chemotherapy starts to work.
  • Plasmapheresis is combined with chemotherapy to control the disease for a longer period of time.

Salvage approach

  • Stem cell transplant is used in patients whose lymphoma relapses or is not responding to other treatments (refractory). [4]

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. ↑ Waldenstrom Macroglobulinemia Treatment Protocols. Medscape 2015. http://emedicine.medscape.com/article/2057687-overview Accessed on November 6 2015
  3. ↑ Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015
  4. ↑ 4.0 4.1 4.2 Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/2057687-overview Accessed on November 11, 2015

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