Management of Waldenstrom macroglobulinemia in 2020.
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Castillo JJ, Treon SP
Management of Waldenstrom macroglobulinemia in 2020.
Hematology Am Soc Hematol Educ Program. 2020 Dec 4;2020(1):372-379. doi: 10.1182/hematology.2020000121.
- PubMed ID
- 33275726 [ View in PubMed]
- Abstract
The management of Waldenstrom macroglobulinemia (WM) has evolved tremendously with recent genomic discoveries that correlate with clinical presentation and could help to tailor treatment approaches. The current diagnosis of WM requires clinicopathological criteria, including bone marrow involvement by lymphoplasmacytic lymphoma cells, a serum immunoglobulin M (IgM) monoclonal paraprotein, and presence of the MYD88 L265P mutation. Once the diagnosis is established, the relationship between the patient's symptoms and WM should be carefully investigated, because therapy should be reserved for symptomatic patients. Bone marrow involvement and serum levels of IgM, albumin, and beta2-microglobulin can be used to estimate the time until treatment initiation. The treatment of WM patients should be highly personalized, and the patient's clinical presentation, comorbidities, genomic profile, and preferences, as well as toxicity of the treatment regimens, should be taken into account. Alkylating agents (bendamustine, cyclophosphamide), proteasome inhibitors (bortezomib, carfilzomib, ixazomib), anti-CD20 monoclonal antibodies (rituximab, ofatumumab), and Bruton tyrosine kinase (BTK) inhibitors (ibrutinib, acalabrutinib, zanubrutinib) are safe and highly effective treatment options in patients with WM. Because novel covalent and noncovalent BTK inhibitors (tirabrutinib, vecabrutinib, LOXO-305, ARQ-531), BCL2 antagonists (venetoclax), and CXCR4-targeting agents (ulocuplumab, mavorixafor) are undergoing clinical development in WM, the future of WM therapy certainly appears bright and hopeful.
DrugBank Data that Cites this Article
- Drug Targets
Drug Target Kind Organism Pharmacological Action Actions Mavorixafor C-X-C chemokine receptor type 4 Protein Humans UnknownAntagonistInhibitorDetails Tirabrutinib Tyrosine-protein kinase BTK Protein Humans UnknownInhibitorDetails Ulocuplumab C-X-C chemokine receptor type 4 Protein Humans YesInhibitorDetails Vecabrutinib Tyrosine-protein kinase BTK Protein Humans UnknownInhibitorDetails Venetoclax Apoptosis regulator Bcl-2 Protein Humans YesAntagonistInhibitorDetails