Risk-tailored treatment of splenic marginal zone lymphoma. Issue 1 (January 2022)

• Record Type: Journal Article
• Authors: Castelli, Roberto; Balzarotti, Monica; Salvi, Emanuele; Simona Rossi, Roberta; Lambertenghi Deliliers, Giorgio; Bergamaschini, Luigi; Gidaro, Antonio
• Is Part Of: Anti-cancer drugs. Volume 33:Issue 1(2022)
• Publication Date: 2022-01
• Subjects: antigen-driven lymphomagenesis -- hepatitis C virus -- indolent lymphoproliferative disease -- marginal zone lymphoma -- splenic marginal zone lymphomas; Antineoplastic agents -- Periodicals; Cancer -- Chemotherapy -- Periodicals; Antineoplastic Agents -- therapeutic use -- Periodicals; Drug Therapy -- Periodicals; 616.994061
• DOI: 10.1097/CAD.0000000000001165
• Languages: English
• ISSNs: 0959-4973

Abstract

Abstract : Splenic marginal zone lymphoma (SMZL) is a rare lymphoproliferative disease involving B-cells and affecting elderly patients. SMZL plague peripheral blood and bone marrow, spleen. Lymph nodes are generally spared. SMZL is due to a protracted antigen stimulation of B lymphocytes and of microenvironment leading B-cell to polyclonal and then oligoclonal/monoclonal growth, promoting lymphoproliferation. Integration of the NOTCH2 and NFk-B signaling has been recently identified as the primary mechanism of neoplastic proliferation in SMZL. In total 20% of cases carry mutations in NOTCH2. Although SMZL has an indolent course, progression to diffuse large B-cell lymphoma occurs in about 10–15% of patients. Establishing the prognosis is a key step in disease management, depending on both individual risk and patients' health status. This review discusses tailored treatment of SMZL patients. Progression risk factors include nodal and extra-nodal involvement, peripheral lymphocytosis, anemia and thrombocytopenia. Patients with two or more score points have a median survival of <5 years. Watch and wait strategy is appropriate in low-risk and asymptomatic patients, whereas treatment of symptomatic patients ranges from splenectomy to rituximab monotherapy or associated with chemotherapy.