Blastoid and pleomorphic mantle cell lymphoma (MCL) are among the worst prognostic, aggressive histology, high-risk variants of MCL, and, in this article, they are presented as blastoid MCL. Blastoid MCL have not been systematically studied, probably due to their rarity. De novo blastoid MCLs have superior outcomes compared with transformed MCL. Compared with classic MCL, extranodal involvement (mainly skin, central nervous system), frequent relapses, and inferior responses to conventional chemoimmunotherapy, BTK inhibitors and venetoclax are frequent in blastoid MCL. KTE-X19 induces excellent response in blastoid MCL. Combinations with novel agents are actively investigated. This article presents a comprehensive review on blastoid MCL in 2020.
Keywords: Aggressive mantle cell lymphoma; BTK; Blastoid; Ibrutinib; Mantle cell lymphoma; Pleomorphic; Transformation; Venetoclax.
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