Waldenström's macroglobulinemia medical therapy

Jump to navigation Jump to search

Waldenström's macroglobulinemia Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Waldenström's macroglobulinemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Bone Marrow Aspiration and Biopsy

Electrophoresis and Immunofixation

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Waldenström's macroglobulinemia medical therapy On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Waldenström's macroglobulinemia medical therapy

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Waldenström's macroglobulinemia medical therapy

CDC on Waldenström's macroglobulinemia medical therapy

Waldenström's macroglobulinemia medical therapy in the news

Blogs on Waldenström's macroglobulinemia medical therapy

Directions to Hospitals Treating Waldenström's macroglobulinemia

Risk calculators and risk factors for Waldenström's macroglobulinemia medical therapy

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

Template:WH Template:WS