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

Revision as of 19:56, 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]

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. [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

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