Lymphoplasmacytic lymphoma medical therapy: Difference between revisions
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* Treat with single agent, rituximab (one cycle only, no maintenance therapy) | * Treat with single agent, rituximab (one cycle only, no maintenance therapy) | ||
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*[[Chemotherapy]] [[drugs]] that may be used with or without [[prednisone]] include: | *[[Chemotherapy]] [[drugs]] that may be used with or without [[prednisone]] include: | ||
Revision as of 22:00, 26 February 2019
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Lymphoplasmacytic lymphoma Microchapters |
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Differentiating Lymphoplasmacytic Lymphoma from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Lymphoplasmacytic lymphoma medical therapy On the Web |
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American Roentgen Ray Society Images of Lymphoplasmacytic lymphoma medical therapy |
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Risk calculators and risk factors for Lymphoplasmacytic lymphoma medical therapy |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]
Overview
Risk stratification determines the protocol of management used for lymphoplasmacytic lymphoma. There is no treatment for asymptomatic lymphoplasmacytic lymphoma. The mainstay of treatment for symptomatic lymphoplasmacytic lymphoma is Rituximab +/- Chemotherapy. Hyperviscosity syndrome is a medical emergency and requires prompt treatment with plasmapheresis.
Medical Therapy
There's no cure for WM/LPL with current therapies. Instead, the treatment goals are to control symptoms and prevent end-organ damage, while maximizing quality of life. There is no standard therapy for the treatment of LPL. While various drugs and combinations have demonstrated to have provided clinical benefit, hence, there are several different options for treating lymphoplasmacytic lymphoma depending on stage of the disease:[1]
Watchful waiting/active surveillance for asymptomatic patients with LPL
There is no treatment for asymptomatic patients with LPL. As LPL develops slowly and may not need to be treated right away, it is monitored by healthcare team every 3-6 months which is known as watchful waiting/active surveillance and treatment is started when symptoms appear, such as hyperviscosity syndrome, or there are signs that the disease is progressing more quickly.[2] 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 patients with LPL
Symptomatic patients with LPL are started on chemotherapy depending on the stage.[3]
- Initial stage of LPL is associated with:
- Neuropathy
- Anemia or cytopenias
- Low-volume nodal involvement
- Asymptomatic splenomegaly
- Late stage of LPL is associated with:
- Adenopathy
- Symptomatic splenomegaly
- Cytopenias
- Hyperviscosity syndrome
- Neuropathy
- Constitutional symptoms
- Men and women with childbearing potential should receive counseling about the potential effect of treatment on their fertility and options for fertility-preserving measures.
| Patient's condition/parameters | How to proceed accordingly |
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Observation |
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Hperviscosity present:
Hyperviscosity absent:
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- 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
| Treatment Regimen[3]
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Drugs | Side effects |
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FR regimen |
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BDR regimen |
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DRC regimen |
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CR regimen |
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IR regimen |
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Hyperviscosity syndrome:
- Lymphoplasmacytic lymphoma complicated with hyperviscosity syndrome is a medical emergency and requires prompt treatment with plasmapheresis.[3]
- 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.
- Plasmapheresis is also used in WM patients with hemolysis.
Initial treatment of Lymphoplasmacytic lymphoma:
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. It works by penetrating the blood brain barrier.[4][5][6][7][8]
- One or more of the following treatments can be given for lymphoplasmacytic lymphoma.
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
In some rare cases, external beam radiation therapy may be required to treat LPL that develops outside of the lymphatic system (called extralymphatic disease).
References
- ↑ Lymphoplasmacytic lymphoma. Canadian Cancer Society 2015. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/lymphoplasmacytic-lymphoma/?region=ab Accessed on November 6 2015
- ↑ Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015
- ↑ 3.0 3.1 3.2 Waldenström's macroglobulinemia: prognosis and management. Blood Cancer Journal (2015)http://www.nature.com/bcj/journal/v5/n3/full/bcj201528a.html Accessed on November 13, 2015
- ↑ O'Neil DS, Francescone MA, Khan K, Bachir A, O'Connor OA, Sawas A (2018). "A Case of Bing-Neel Syndrome Successfully Treated with Ibrutinib". Case Rep Hematol. 2018: 8573105. doi:10.1155/2018/8573105. PMC 6136466. PMID 30228918.
- ↑ Minnema MC, Kimby E, D'Sa S, Fornecker LM, Poulain S, Snijders TJ; et al. (2017). "Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome". Haematologica. 102 (1): 43–51. doi:10.3324/haematol.2016.147728. PMC 5210231. PMID 27758817.
- ↑ Tallant A, Selig D, Wanko SO, Roswarski J (2018). "First-line ibrutinib for Bing-Neel syndrome". BMJ Case Rep. 2018. doi:10.1136/bcr-2018-226102. PMID 30279255.
- ↑ Cabannes-Hamy A, Lemal R, Goldwirt L, Poulain S, Amorim S, Pérignon R; et al. (2016). "Efficacy of ibrutinib in the treatment of Bing-Neel syndrome". Am J Hematol. 91 (3): E17–9. doi:10.1002/ajh.24279. PMID 26689870.
- ↑ Mason C, Savona S, Rini JN, Castillo JJ, Xu L, Hunter ZR; et al. (2017). "Ibrutinib penetrates the blood brain barrier and shows efficacy in the therapy of Bing Neel syndrome". Br J Haematol. 179 (2): 339–341. doi:10.1111/bjh.14218. PMID 27409073.