4.2 Article

Progressive leukemic non-nodal mantle cell lymphoma associated with deletions of TP53, ATM, and/or 13q14

Journal

ANNALS OF DIAGNOSTIC PATHOLOGY
Volume 18, Issue 4, Pages 214-219

Publisher

ELSEVIER SCIENCE INC
DOI: 10.1016/j.anndiagpath.2014.03.006

Keywords

Leukemic mantle cell lymphoma; Non-nodal mantle cell lymphoma; Thp53 alteration; ATM deletion

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Funding

  1. National Institutes of Health (NIH) [CA109335, CA122105]
  2. Dwoskin Family Foundation
  3. Redo Family Foundation

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Leukemic, non-nodal mantle cell lymphoma (MCL) is a relatively indolent disease characterized by asymptomatic leukemic presentation, non-nodal disease distribution, and slow disease progression, particularly in comparison to that of classic nodal MCL. We studied 3 cases of leukemic, non-nodal MCL in which TP53, ATM, and/or 13q14 deletions were identified. All three patients had disease progression leading to treatment requirements in two of the patients at 5 and 18 months after initial diagnosis. The third patient also clinically progressed 25 months after initial diagnosis but was lost to follow up despite recommendation for initiation of therapy. We present these cases as potential evidence that while leukemic non-nodal MCL is typically an indolent disease compared to classically defined mantle cell lymphoma, cytogenetic heterogeneity exists and cases with TP53, ATM, and/or 13q14 deletions may have a relatively aggressive clinical course. (C) 2014 Elsevier Inc. All rights reserved.

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