<i>DUSP22</i> rearrangement is associated with a distinctive immunophenotype but not outcome in patients with systemic ALK-negative anaplastic large cell lymphoma
Lianqun Qiu,
Guilin Tang,
Shaoying Li,
Francisco Vega,
Pei Lin,
Sa A. Wang,
Wei Wang,
Swaminathan P. Iyer,
Luis Malpica,
Roberto N. Miranda,
Sergej Konoplev,
Zhenya Tang,
Hong Fang,
L. Jeffrey Medeiros,
Jie Xu
Affiliations
Lianqun Qiu
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Guilin Tang
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Shaoying Li
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Francisco Vega
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Pei Lin
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Sa A. Wang
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Wei Wang
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Swaminathan P. Iyer
Department of Lymphoma/Myeloma, The University of Texas MD Anderson Cancer Center
Luis Malpica
Department of Lymphoma/Myeloma, The University of Texas MD Anderson Cancer Center
Roberto N. Miranda
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Sergej Konoplev
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Zhenya Tang
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Hong Fang
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
L. Jeffrey Medeiros
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
Jie Xu
Department of Hematopathology, The University of Texas MD Anderson Cancer Center
DUSP22 rearrangement (R) has been associated with a favorable outcome in systemic ALK-negative anaplastic large cell lymphoma (ALCL). However, a recent study found that patients with DUSP22-R ALK-negative ALCL have a poorer prognosis than was reported initially. In this study, we compared the clinicopathological features and outcomes of patients with ALKnegative ALCL with DUSP22-R (n=22) versus those without DUSP22-R (DUSP22-NR; n=59). Patients with DUSP22-R ALCL were younger than those with DUSP22-NR neoplasms (P=0.049). DUSP22-R ALK-negative ALCL cases were more often positive for CD15, CD8, and less frequently expressed pSTAT3Tyr705, PD-L1, granzyme B and EMA (all P0.05), but was significantly shorter than that of the patients with ALK-positive ALCL (median overall survival 53 months vs. undefined, P=0.005). Five-year overall survival rates were 40% for patients with DUSP22-R ALCL versus 82% for patients with ALK-positive ALCL. We conclude that DUSP22-R neoplasms represent a distinctive subset of ALK-negative ALCL. However, in this cohort DUSP22-R was not associated with a better clinical outcome. Therefore, we suggest that current treatment guidelines for this subset of ALK-negative ALCL patients should not be modified at present.
