@article{Qiu_Tang_Li_Vega_Lin_Wang_Wang_Iyer_Malpica_Miranda_Konoplev_Tang_Fang_Medeiros_Xu_2023, place={Pavia, Italy}, title={<i>DUSP22</i> rearrangement is associated with a distinctive immunophenotype but not outcome in patients with systemic ALK-negative anaplastic large cell lymphoma}, volume={108}, url={https://haematologica.org/article/view/haematol.2022.281222}, DOI={10.3324/haematol.2022.281222}, abstractNote={<p><em>DUSP22</em> 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 <em>DUSP22</em>-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 <em>DUSP22</em>-R (n=22) versus those without <em>DUSP22</em>-R (DUSP22-NR; n=59). Patients with <em>DUSP22</em>-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 pSTAT3<sup>Tyr705</sup>, PD-L1, granzyme B and EMA (all P&lt;0.05). TP63 rearrangement (TP63-R) was detected in three of the 66 (5%) ALK-negative ALCL cases tested and none of these cases carried the <em>DUSP22</em>-R. Overall survival of patients with <em>DUSP22</em>-R ALCL was similar to that of the patients with <em>DUSP22</em>-NR neoplasms regardless of International Prognostic Index score, stage, age, or stem cell transplantation status (all P&gt;0.05), 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 <em>DUSP22</em>-R ALCL versus 82% for patients with ALK-positive ALCL. We conclude that <em>DUSP22</em>-R neoplasms represent a distinctive subset of ALK-negative ALCL. However, in this cohort <em>DUSP22</em>-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.</p&gt;}, number={6}, journal={Haematologica}, author={Qiu, Lianqun and Tang, Guilin and Li, Shaoying and Vega, Francisco and Lin, Pei and Wang, Sa A. and Wang, Wei and Iyer, Swaminathan P. and Malpica, Luis and Miranda, Roberto N. and Konoplev, Sergej and Tang, Zhenya and Fang, Hong and Medeiros, L. Jeffrey and Xu, Jie}, year={2023}, month={Jun.}, pages={1604-1615} }