Curr. Treat. Options in Oncol. (2019) 20:85 DOI 10.1007/s11864-019-0684-8 Lymphoma (DO Persky, Section Editor) Leukemic Non-nodal Mantle Cell Lymphoma: Diagnosis and Treatment Akriti Gupta Jain, MD1 Chung-Che Chang, MD, PhD2 Sarfraz Ahmad, PhD3 Shahram Mori, MD, PhD4,* Address 1Department of Internal Medicine Residency Program, AdventHealth Cancer In- stitute, Orlando, FL, USA 2Department of Pathology & Laboratory Medicine, AdventHealth Cancer Institute, Orlando, FL, USA 3Department of Gynecologic Oncology, AdventHealth Cancer Institute, Orlando, FL, USA *,4Blood and Marrow Transplant Center, AdventHealth Cancer Institute, 2415 N. Orange Ave., Suite 601, Orlando, FL, 32804, USA Email:
[email protected] * Springer Science+Business Media, LLC, part of Springer Nature 2019 This article is part of the Topical Collection on Lymphoma Keywords Leukemic I Mantle cell lymphoma I Non-nodal I P53 gene mutation I Diagnosis I Treatment Opinion statement Mantle cell lymphoma (MCL) encompasses nearly 6% of all the non-Hodgkin lymphomas. It is considered an incurable neoplastic process arising from B cells. The cytogenetic abnormality t(11;14) (q13; q32) leading to cyclin D1 overexpression is the sentinel genetic event and provides an exceptional marker for diagnosis. MCL is generally consid- ered to have an aggressive course as compared with other indolent lymphomas with traditionally reported median survival of 3–5 years. According to the 2016 WHO classifi- cation, there are two major known variants of MCL: classical which affects the lymph nodes and extra nodal sites and leukemic non-nodal MCL (L-NN-MCL) which characteristically involves the bone marrow, peripheral blood, and the spleen.