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.

Risk-tailored treatment of splenic marginal zone lymphoma / Castelli, R.; Balzarotti, M.; Salvi, E.; Simona Rossi, R.; Lambertenghi Deliliers, G.; Bergamaschini, L.; Gidaro, A.. - In: ANTI-CANCER DRUGS. - ISSN 1473-5741. - 33:1(2022), pp. e36-e42. [10.1097/CAD.0000000000001165]

Risk-tailored treatment of splenic marginal zone lymphoma

Castelli R.;
2022-01-01

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.
2022
Risk-tailored treatment of splenic marginal zone lymphoma / Castelli, R.; Balzarotti, M.; Salvi, E.; Simona Rossi, R.; Lambertenghi Deliliers, G.; Bergamaschini, L.; Gidaro, A.. - In: ANTI-CANCER DRUGS. - ISSN 1473-5741. - 33:1(2022), pp. e36-e42. [10.1097/CAD.0000000000001165]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11388/254760
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