Progressive leukemic non-nodal mantle cell lymphoma associated with deletions of TP53, ATM, and/or 13q14.
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.
Division of Hematopathology, Department of Pathology, University of Miami, Miami, FL.
Jennifer
J
Chapman-Fredricks
Division of Hematology-Oncology, Department of Medicine, Sylvester Comprehensive Cancer Center, Miami, FL.
Jose
J
Sandoval-Sus
Division of Hematopathology, Department of Pathology, University of Miami, Miami, FL.
Francisco
F
Vega
Division of Hematology-Oncology, Department of Medicine, Sylvester Comprehensive Cancer Center, Miami, FL; Department of Molecular and Cellular Pharmacology, University of Miami, Miami, FL.
Izidore S
IS
Lossos
D000368:Aged; D064007:Ataxia Telangiectasia Mutated Proteins / Q000235:genetics*; D002882:Chromosomes, Human, Pair 13 / Q000235:genetics*; D005260:Female; D005434:Flow Cytometry; D017353:Gene Deletion; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D020522:Lymphoma, Mantle-Cell / Q000235:genetics* / Q000473:pathology*; D016159:Tumor Suppressor Protein p53 / Q000235:genetics*
C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53; C576324:ATM protein, human; D064007:Ataxia Telangiectasia Mutated Proteins
ATM deletion; Leukemic mantle cell lymphoma; Non-nodal mantle cell lymphoma; TPp53 alteration