Clinicopathologic features and management of blastoid variant of mantle cell lymphoma

Abstract

The blastoid variant of mantle cell lymphoma (MCL), which accounts for less than one-third of MCL, may arise de novo or as a transformation from the classical form of MCL. Blastoid variant, which predominantly involves men in their sixth decade, has frequent extranodal involvement (40–60%), stage IV disease (up to 85%) and central nervous system (CNS) involvement. Diagnosis relies on morphological features and is challenging. Immunophenotyping may display CD23 and CD10 positivity and CD5 negativity in a subset. Genetic analysis demonstrates an increased number of complex genetic alterations. Blastoid variant responds poorly to conventional chemotherapy and has a short duration of response. Although the optimal therapy remains to be established, CNS prophylaxis and the use of aggressive immunochemotherapy followed by autologous stem cell transplant may prolong the remission rate and survival. Further studies are crucial to expand our understanding of this disease entity and improve the clinical outcome.

Keywords::

• Mantle cell lymphoma
• blastoid variant
• genetic alteration
• central nervous system involvement
• intensified chemotherapy
• high dose chemotherapy
• autologous stem cell transplant

Acknowledgement

The authors would like to extend their gratitude to Dr. Guiyuan Li, MD, Department of Pathology and Microbiology at the University of Nebraska Medical Center for providing the pathology images used in this article.

Potential conflict of interest

Disclosure forms provided by the authors are available with the full text of this article at www.informahealthcare.com/lal.

Supplementary material available online

Supplementary Tables I showing mantle cell lymphoma studies including patients with blastoid variant (cites refs. [93–95])