Emerging therapeutic options for Waldenström macroglobulinemia/lymphoplasmacytic lymphoma

Abstract

Lymphoplasmacytic lymphoma is an indolent B-cell, non-Hodgkin lymphoma (NHL), the majority of which are characterized by production of a monoclonal immunoglobulin M (IgM) protein and are known as Waldenström macroglobulinemia. Identification of highly recurrent activating somatic mutation in MYD88 has improved our understanding of the pathogenesis of Waldenström macroglobulinemia and has therapeutic implications. Here, we review novel therapeutic agents in Waldenström macroglobulinemia/lymphoplasmacytic lymphoma, which have emerged in the past decade and discuss their comparative efficacy and safety, with emphasis on a Bruton's tyrosine kinase (BTK) inhibitor, which has been recently approved by the US FDA, specifically for Waldenström macroglobulinemia/lymphoplasmacytic lymphoma. Future research should focus on identifying targeted agents against activating mutations and long-term data for currently available novel agents should be critically evaluated, both in treatment-naïve and in relapsed/refractory settings.

An uncommon case of lymphoplasmacytic lymphoma in cerebellopontine angle region

“…The chemotherapy regimen is cyclophosphamide + doxorubicin + vincristine + prednison (CHOP) or cyclophosphamide, vincristine, and prednisone, with rituximab—a monoclonal antibody widely used against B cell lymphoma. [19] Patients who received rituximab along with CHOP reportedly had significantly higher rates of overall response than patients who only received the traditional regimen.…”

Diagnosis and Management of Oncologic Emergencies

“…The mainstays of therapy are decreasing serum viscosity through IV fluid resuscitation, plasmapheresis, or leukopheresis 63–67. Phlebotomy (e.g., polycythemia) or even urgent chemotherapy (e.g., acute leukemia) may also be indicated 63–67. Evidence for these interventions suggests they do not alter the course of the disease but rather help with symptom alleviation.…”