Lymphoplasmacytic lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Synonyms and keywords: Waldenstrom's macroglobulinemia; Waldenstrom's disease; Primary macroglobulinemia; Hyperviscosity syndrome; Lymphoplasmacytoid lymphoma

Diagnosis

• Not all the diagnostic tests mentioned are performed in a WM patient. A doctor takes into account the following factors before choosing diagnostic tests in a particular patient:
  • Suspected type of cancer.
  • Signs and symptoms.
  • Age.
  • Medical condition of the patient.
  • Results of earlier medical tests.

Treatment

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.

Surgery

Stem cell transplant is usually reserved for patients with either relapse or refractory Waldenström's macroglobulinemia.

Primary Prevention

Primary prevention of Waldenström macroglobulinemia depends on the type of risk factor causing the disease.

Modifiable risk factors

• Hepatitis C

Non-modifiable risk factors

• Age > 50
• Male gender
• Race - White
• Hereditary- Monoclonal gammopathy of undetermined significance

Secondary Prevention

There are no established measures for the secondary prevention of Waldenström's macroglobulinemia.

One or more of the following treatments can be given for lymphoplasmacytic lymphoma.

Watchful waiting

Watchful waiting (also called active surveillance) may be offered for lymphoplasmacytic lymphoma because it develops slowly and may not need to be treated right away. The healthcare team will carefully monitor the person with lymphoplasmacytic lymphoma and start treatment when symptoms appear, such as hyperviscosity syndrome, or there are signs that the disease is progressing more quickly.

Chemotherapy

• People with lymphoplasmacytic lymphoma who have symptoms or hyperviscosity syndrome are usually given chemotherapy. Chemotherapy drugs that may be used with or without prednisone include:
  • Chlorambucil (Leukeran)
  • Fludarabine (Fludara)
  • Bendamustine (Treanda)
  • Cyclophosphamide (Cytoxan, Procytox)

• Combinations of chemotherapy drugs that may be used include:
  • DRC – dexamethasone (Decadron, Dexasone), rituximab (Rituxan) and cyclophosphamide
  • BRD – bortezomib (Velcade) and rituximab, with or without dexamethasone
  • CVP – cyclophosphamide, vincristine (Oncovin) and prednisone
  • R-CVP – CVP with rituximab
  • Thalidomide (Thalomid) and rituximab

Targeted therapy

• Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.
• Targeted therapy drugs used alone or in combination to treat lymphoplasmacytic lymphoma include rituximab, bortezomib and ibrutinib (Imbruvica).

Immunotherapy

• Immunotherapy works by stimulating, boosting, restoring or acting like the body’s immune system to create a response against cancer cells. Immunomodulatory drugs are a type of immunotherapy that interferes with the growth and division of cancer cells.
• Thalidomide is a type of immunomodulatory drug that may be used to treat lymphoplasmacytic lymphoma.

Radiation therapy

External beam radiation therapy may be used to treat lymphoplasmacytic lymphoma that develops outside of the lymphatic system (called extralymphatic disease), but this is rare.

Stem cell transplant

• Some people with lymphoplasmacytic lymphoma may be offered a stem cell transplant.
• It may be used if the lymphoma comes back (recurs) after treatment or doesn’t respond to other treatments (called refractory disease).
• Many people with lymphoplasmacytic lymphoma are older or may not be in good health, so a stem cell transplant may not be a good treatment option for them.

Read more: http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/more-types-of-nhl/lymphoplasmacytic-lymphoma/?region=on#ixzz5eb6iT7G6