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:::*symptomatic cryoglobulinemia
:::*symptomatic cryoglobulinemia
:::*Thrombocytopenia
:::*Thrombocytopenia


===Hyperviscosity syndrome===
===Hyperviscosity syndrome===

Revision as of 19:17, 16 November 2015

Waldenström's macroglobulinemia Microchapters

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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. Watchful waiting is recommended for asymptomatic Waldenström's macroglobulinemia. Symptomatic Waldenström's macroglobulinemia is treated with Rituximab +/- Chemotherapy.[1]

Medical Therapy

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

References

  1. 1.0 1.1 1.2 1.3 Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/2057687-overview Accessed on November 11, 2015
  2. 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
  3. Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 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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