ALK-negative anaplastic large cell lymphoma with DUSP22 rearrangement has distinctive disease characteristics with better progression-free survival: a LYSA study.
- Sibon, D.
- Bisig, B.
- Bonnet, C.
- Poullot, E.
- Bachy, E.
- Cavalieri, D.
- Fataccioli, V.
- Bregnard, C.
- Drieux, F.
- Bruneau, J.
- Lemonnier, F.
- Dupuy, A.
- Bossard, C.
- Parrens, M.
- Bouabdallah, K.
- Ketterer, N.
- Berthod, G.
- Cairoli, A.
- Damaj, G.
- Tournilhac, O.
- Jais, J.P.
- Gaulard, P.
- De Leval, L.
Publication date
December 2022
DOI Publisher
Ferrata Storti Foundation (Haematologica)Abstract
ALK-negative anaplastic large cell lymphoma (ALCL) comprises subgroups harboring rearrangements of DUSP22 (DUSP22-R) or TP63 (TP63-R). Two studies respectively reported 90% and 40% 5-year overall survival (OS) in 21 and 12 DUSP22-R/TP63-not rearranged (NR) patients, making the prognostic impact of DUSP22-R unclear. Here, 104 newly diagnosed ALK-negative ALCL patients (including 37 from first-line clinical trials) from the LYSA TENOMIC database were analyzed by break-apart FISH assays for DUSP22-R and TP63-R. There were 47/104 (45%) DUSP22-R and 2/93 (2%) TP63-R cases, including one DUSP22-R/TP63-R. DUSP22-R tumors showed more frequent CD3 expression (62% versus 35%, P=0.01), and less commonly a cytotoxic phenotype (27% versus 82%; P
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