Waldenström's macroglobulinemia medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2] Roukoz A. Karam, M.D.[3]

Overview

Risk stratification determines the protocol of management used for Waldenström macroglobulinemia. There is no treatment for asymptomatic Waldenström macroglobulinemia. The mainstay of treatment for symptomatic Waldenström 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 macroglobulinemia. Asymptomatic waldenström's macroglobulinemia can be monitored every 3-6 months.

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: Rituximab-based therapy, as below.

• Late stage of Waldenström's macroglobulinemia associated with:

• Adenopathy
• Symptomatic splenomegaly
• Cytopenias
• Hyperviscosity syndrome
• Neuropathy
• Constitutional symptoms

Treatment:

Treatment Regimen	Drugs	Side effects
CHOP-R regimen	Cyclophosphamide Doxorubicin Vincristine Prednisone Rituximab	Nausea Alopecia Granulocytopenia Cardiotoxicity Mucositis
Ibrutinib	Ibrutinib	Fatigue Cytopenia Bleeding Atrial fibrillation Opportunistic infection
Rituximab	Rituximab	Infusion related reaction Hepatitis B reaction Progressive multifocal leukoencephaloptahy
FR regimen	Fludarabine Rituximab	Neutropenia (63%) Thrombocytopenia Pneumonia
BDR regimen	Bortezomib Dexamethasone Rituximab	Peripheral neuropathy - reversible in 61% of patients Infections
DRC regimen	Dexamethasone Rituximab Cyclophosphamide	Neutropenia
CR regimen	Cladribine Rituximab	Anemia Neurological symptoms Symptomatic cryoglobulinemia Thrombocytopenia
IR regimen	Ibrutinib Rituximab	Anemia Neurological symptoms Symptomatic cryoglobulinemia Thrombocytopenia Atrial fibrillation

Hyperviscosity syndrome

• Waldenström 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.