Clinicopathologic features of systemic ALK-negative anaplastic large cell lymphoma with TP53 deletion

Objectives: In this study, we illustrated the significance of TP53 deletion (D) in systemic ALK-negative anaplastic large cell lymphoma (ALCL), an area that remains unknown. Methods: We evaluated TP53-D by fluorescence in situ hybridization in 66 patients with systemic ALK-negative ALCL and compared the results with clinicopathologic features. Results: TP53-D was identified in 32 (48%) cases. Peripheral blood involvement occurred exclusively in the TP53-D group compared with the TP53–not deleted (ND) group (50% vs 0%, P = .01). TP53-D cases more frequently showed p53 overexpression by immunohistochemistry than TP53-ND cases (P = .03). Among the International Prognostic Index (IPI) score, TP53-D, DUSP22 rearrangement (DUSP22-R), and stem cell transplantation status, for the entire cohort, only a low IPI score (<3) was associated with superior overall survival (P = .04), while none of these factors affected progression-free survival (PFS). In TP53-ND patients, low IPI and DUSP22-R were associated with significantly longer PFS (P = .04 and P = .02); these associations were absent in TP53-D patients. Conclusions: TP53-D is common in systemic ALK-negative ALCL and is associated with leukemic disease and p53 overexpression. TP53-D did not directly affect survival, but it negated the favorable impact of low IPI and DUSP22-R on PFS, suggesting its potential value as an adverse prognostic marker.