Approximately 10%-20% of patients with MCL present with an indolent form of the disease, including those with smoldering or asymptomatic non-nodal disease. Patients with this variant do not exhibit high-risk clinical or biologic features and often present with circulating disease, marrow involvement, and splenomegaly. SOX-11 is typically negative in this variant. Some patients survive > 7-10 years, and immediate treatment with systemic therapy may not be needed; a "watch and wait" strategy may be an option. Patients with non-nodal disease can acquire TP53 mutations, manifested by rapid progression of disease.
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Cite this: Ann S. LaCasce. Fast Five Quiz: Non-Hodgkin's Lymphoma: Mantle Cell Lymphoma Practice Essentials - Medscape - May 22, 2023.
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