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. There is no treatment for asymptomatic waldenström's macroglobulinemia. The mainstay of treatment for symptomatic waldenström's macroglobulinemia is Rituximab +/- Chemotherapy. Hyperviscosity syndrome is a medical emergency and requires prompt treatment with plasmapheresis.

Medical Therapy

There are several different options for treating waldenström macroglobulinemia depending on stage of the disease.

Asymptomatic/Smoldering Waldenström's Macroglobulinemia

There is no treatment for asymptomatic waldenström's macroglobulinemia. Asymptomatic waldenström's macroglobulinemia 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

• Waldenström's macroglobulinemia complicated with hyperviscosity syndrome is a medical emergency and requires prompt treatment with plasmapheresis.
• Plasmapheresis temporarily lowers IgM levels by removing some of the abnormal IgM from the blood, which makes blood thinner.
• Plasmapheresis is usually given until chemotherapy starts to work.
• Plasmapheresis is combined with chemotherapy to control the disease for a longer period of time.