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. The mainstay of therapy for asymptomatic Waldenström's macroglobulinemia is watchful waiting. Symptomatic Waldenström's macroglobulinemia is treated with Rituximab +/- Chemotherapy.

Medical Therapy

There are several different options for treating Waldenström Macroglobulinemia depending on the disease stage.

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:

Treatment Regimen	Drugs	Side effects
CHOP-R regimen	Cyclophosphamide Doxorubicin Vincristine Prednisone Rituximab	Nausea Alopecia Granulocytopenia
Ibrutinib	Ibrutinib	Fatigue Cytopenia
Rituximab	Rituximab	Infusion related reaction Infections
FR regimen	Fludarabine Rituximab	Neutropenia (63%) Thrombocytopenia Pneumonia
BDR regimen	Bortezomib Dexamethasone Rituximab	Peripheral Neuropathy - reversible in 61% of patients
DRC regimen	Dexamethasone Rituximab Cyclophosphamide	Neutropenia
CR regimen	Cladribine Rituximab	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.