Waldenström's macroglobulinemia medical therapy: Difference between revisions

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*Constitutional symptoms
*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>
'''Treatment regimen used:'''<ref name="PHARM">Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/2057687-overview Accessed on November 11, 2015</ref><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>


'''CHOP-R regimen:'''
'''CHOP-R regimen:'''
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*Rituximab 375 mg/m2 IV day 1;
*Rituximab 375 mg/m2 IV day 1;
*Primary: Repeat 21 day cycle for 6-8 cycles
*Primary: Repeat 21 day cycle for 6-8 cycles
::*'''Adverse Effect:'''
:::*Nausea
:::*Alopecia
:::*Granulocytopenia


'''Ibrutinib''' 420 mg PO once daily until disease progression
'''Ibrutinib''' 420 mg PO once daily until disease progression
::*'''Adverse Effect:'''
:::*Fatigue
:::*Cytopenia


'''Rituximab''' 375 mg/m2 IV once weekly x 4 weeks
'''Rituximab''' 375 mg/m2 IV once weekly x 4 weeks
::*'''Adverse Effect:'''
:::*Infusion related reaction
:::*Infections


'''Fludarabine/Rituximab regimen:'''
'''Fludarabine/Rituximab regimen:'''
Line 48: Line 58:
*Rituximab 375 mg/m2 IV day 1;
*Rituximab 375 mg/m2 IV day 1;
*Repeat 28 day cycle for 4-6 cycles
*Repeat 28 day cycle for 4-6 cycles
::*'''Adverse Effect:'''
:::*Neutropenia (63%)
:::*Thrombocytopenia
:::*Pneumonia


'''FCR regimen:'''
'''FCR regimen:'''
Line 55: Line 69:
*Primary: Repeat 28 day cycle for 4-6 cycles
*Primary: Repeat 28 day cycle for 4-6 cycles
*May also be given with mitoxantrone 10 mg/m2 on day 1
*May also be given with mitoxantrone 10 mg/m2 on day 1
::*'''Adverse Effect:'''
:::*Neurtropenia


'''BR regimen:'''
'''BR regimen:'''
Line 60: Line 76:
*Rituximab 375 mg/m2 IV day 1
*Rituximab 375 mg/m2 IV day 1
*Primary: Repeat 21 day cycle for 6 cycles
*Primary: Repeat 21 day cycle for 6 cycles
::*'''Adverse Effect:'''
:::*Myelosupression
:::*Neutropenia
:::*Thrombocytopenia


'''BDR regimen:'''
'''BDR regimen:'''
Line 66: Line 86:
*Rituximab 375 mg/m2 IV day 11
*Rituximab 375 mg/m2 IV day 11
*Primary: Repeat 21 day cycle for 4 cycles  
*Primary: Repeat 21 day cycle for 4 cycles  
::*'''Adverse Effect:'''
:::*Peripheral neuropathy - reversible in 61% of patients


'''DRC regimen:'''
'''DRC regimen:'''
Line 72: Line 94:
*Cyclophosphamide 100 mg/m2 PO BID days 1-5
*Cyclophosphamide 100 mg/m2 PO BID days 1-5
*Primary: Repeat 21 day cycle for 6 cycles
*Primary: Repeat 21 day cycle for 6 cycles
::*'''Adverse Effect:'''
:::*Neutropenia
:::*Rituximab associated toxicity


'''CR regimen:'''
'''CR regimen:'''
Line 77: Line 102:
*Rituximab 375 mg/m2 IV day 1
*Rituximab 375 mg/m2 IV day 1
*Primary: Repeat 28 day cycle for 4 cycles
*Primary: Repeat 28 day cycle for 4 cycles
::*'''Adverse Effect:'''
:::*Anemia
:::*Neurological symptoms
:::*symptomatic cryoglobulinemia
:::*Thrombocytopenia


===Hyperviscosity syndrome===
===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 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.

Revision as of 20:50, 13 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

Risk stratification determines the protocol of management used for Waldenström's macroglobulinemia patients.

Medical Therapy

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

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'.[2]

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:[3][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
  • Adverse Effect:
  • Nausea
  • Alopecia
  • Granulocytopenia

Ibrutinib 420 mg PO once daily until disease progression

  • Adverse Effect:
  • Fatigue
  • Cytopenia

Rituximab 375 mg/m2 IV once weekly x 4 weeks

  • Adverse Effect:
  • Infusion related reaction
  • Infections

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
  • Adverse Effect:
  • Neutropenia (63%)
  • Thrombocytopenia
  • Pneumonia

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
  • Adverse Effect:
  • Neurtropenia

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
  • Adverse Effect:
  • Myelosupression
  • Neutropenia
  • Thrombocytopenia

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
  • Adverse Effect:
  • Peripheral neuropathy - reversible in 61% of patients

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
  • Adverse Effect:
  • Neutropenia
  • Rituximab associated toxicity

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
  • Adverse Effect:
  • Anemia
  • Neurological symptoms
  • symptomatic cryoglobulinemia
  • Thrombocytopenia


Hyperviscosity syndrome

  • Plasmapheresis is recommended emergent treatment option for patients with Waldenström Macroglobulinemia who develop hyperviscosity symptoms.[3]
  • 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). [3]

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. Medscape (2015)http://emedicine.medscape.com/article/2057687-overview Accessed on November 11, 2015
  4. ↑ 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

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