ALK-negative anaplastic large cell lymphoma with <i>DUSP22</i> rearrangement has distinctive disease characteristics with better progression-free survival: a LYSA study Article Swipe
David Sibon
,
Bettina Bisig
,
Christophe Bonnet
,
Elsa Poullot
,
Emmanuel Bachy
,
Doriane Cavalieri
,
Virginie Fataccioli
,
Cloe Bregnard
,
Fanny Drieux
,
Julie Bruneau
,
François Lemonnier
,
Aurélie Dupuy
,
Céline Bossard
,
Marie Parrens
,
Krimo Bouabdallah
,
Nicolas Ketterer
,
Grégoire Berthod
,
Anne Cairoli
,
Gandhi Damaj
,
Olivier Tournilhac
,
Jean‐Philippe Jaïs
,
Philippe Gaulard
,
Laurence de Leval
·
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.3324/haematol.2022.281442
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.3324/haematol.2022.281442
ALK-negative anaplastic large cell lymphoma (ALCL) comprises subgroups harboring rearrangements of DUSP22 (DUSP22- R) or TP63 (TP63-R). Two studies reported 90% and 40% 5-year overall survival (OS) rates in 21 and 12 DUSP22-R/TP63- not rearranged (NR) patients, respectively, 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 fluorescence in situ hybridization 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 case. DUSP22-R tumors more frequently showed CD3 expression (62% vs. 35%, P=0.01), and less commonly a cytotoxic phenotype (27% vs. 82%; P<0.001). At diagnosis, DUSP22- R ALCL patients more frequently had bone involvement (32% vs. 13%, P=0.03). The patient with DUSP22-R/TP63-R ALCL had a rapidly fatal outcome. After a median follow-up of 4.9 years, 5-year progression-free survival (PFS) and OS rates of 84 patients without TP63-R treated with curative-intent anthracycline-based chemotherapy were 41% and 53%, respectively. According to DUSP22 status, 5-year PFS was 57% for 39 DUSP22-R versus 26% for 45 triple-negative (DUSP22-NR/TP63-NR/ALK-negative) patients (P=0.001). The corresponding 5-year OS rates were 65% and 41%, respectively (P=0.07). In multivariate analysis, performance status and DUSP22 status significantly affected PFS, and distinguished four risk groups, with 4-year PFS and OS ranging from 17% to 73% and 21% to 77%, respectively. Performance status but not DUSP22 status influenced OS. The use of brentuximab vedotin in relapsed/refractory patients improved OS independently of DUSP22 status. Our findings support the biological and clinical distinctiveness of DUSP22- R ALK-negative ALCL. Its relevance to outcome in patients receiving frontline brentuximab vedotin remains to be determined.
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Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.3324/haematol.2022.281442
- https://haematologica.org/article/download/haematol.2022.281442/75103
- OA Status
- gold
- Cited By
- 25
- References
- 40
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W4310567981
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
-
https://openalex.org/W4310567981Canonical identifier for this work in OpenAlex
- DOI
-
https://doi.org/10.3324/haematol.2022.281442Digital Object Identifier
- Title
-
ALK-negative anaplastic large cell lymphoma with <i>DUSP22</i> rearrangement has distinctive disease characteristics with better progression-free survival: a LYSA studyWork title
- Type
-
articleOpenAlex work type
- Language
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enPrimary language
- Publication year
-
2022Year of publication
- Publication date
-
2022-12-01Full publication date if available
- Authors
-
David Sibon, Bettina Bisig, Christophe Bonnet, Elsa Poullot, Emmanuel Bachy, Doriane Cavalieri, Virginie Fataccioli, Cloe Bregnard, Fanny Drieux, Julie Bruneau, François Lemonnier, Aurélie Dupuy, Céline Bossard, Marie Parrens, Krimo Bouabdallah, Nicolas Ketterer, Grégoire Berthod, Anne Cairoli, Gandhi Damaj, Olivier Tournilhac, Jean‐Philippe Jaïs, Philippe Gaulard, Laurence de LevalList of authors in order
- Landing page
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https://doi.org/10.3324/haematol.2022.281442Publisher landing page
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https://haematologica.org/article/download/haematol.2022.281442/75103Direct link to full text PDF
- Open access
-
YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
- OA URL
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https://haematologica.org/article/download/haematol.2022.281442/75103Direct OA link when available
- Concepts
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Anaplastic large-cell lymphoma, Medicine, Internal medicine, Lymphoma, Oncology, Cancer researchTop concepts (fields/topics) attached by OpenAlex
- Cited by
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25Total citation count in OpenAlex
- Citations by year (recent)
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2025: 8, 2024: 7, 2023: 9, 2022: 1Per-year citation counts (last 5 years)
- References (count)
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40Number of works referenced by this work
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10Other works algorithmically related by OpenAlex
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