Waldenström's macroglobulinemia medical therapy

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.

Medical Therapy

There are several different options for Waldenström Macroglobulinemia.

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'.

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:

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.
• 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).