Lymphoplasmacytic lymphoma medical therapy

Lymphoplasmacytic lymphoma Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

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. Active surveillance includes monitoring of the following laboratory parameters:

• Complete blood count (CBC) with differential
• Complete metabolic panel (CMP)
• Immunoglobulin levels in the serum (quantitative)
• Serum protein electrophoresis

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
• Asymptomatic splenomegaly

• Late stage of Waldenström's macroglobulinemia associated with:

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

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 multi-focal 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.

Drug of choice for Bing-Neel Syndrome

Many recent studies have shown to be Ibrutinib (560mg), an oral Bruton's tyrosine kinase inhibitor, with or without concurrent Rituximab, as a drug of choice for treatment of Bing-Neel syndrome.