Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions: reevaluation of the defining characteristics in a registry-based cohort
Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions: reevaluation of the defining characteristics in a registry-based cohort
In a registry-based analysis of 135 patients with “myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions” (MLN-TK; FIP1L1::PDGFRA, n = 78; PDGFRB, diverse fusions, n = 26; FGFR1, diverse, n = 9; JAK2, diverse, n = 11; ETV6::ABL1, n = 11), we sought to evaluate the disease-defining characteristics. In 81/135 (60%) evaluable patients, hypereosinophilia (>1.5 × 10 9/l) was observed in 40/44 (91%) FIP1L1::PDGFRA and 7/7 (100%) ETV6::ABL1 positive patients but only in 13/30 (43%) patients with PDGFRB, FGFR1, and JAK2 fusion genes while 9/30 (30%) patients had no eosinophilia. Monocytosis >1 × 10 9/l was identified in 27/81 (33%) patients, most frequently in association with hypereosinophilia (23/27, 85%). Overall, a blast phase (BP) was diagnosed in 38/135 (28%) patients (myeloid, 61%; lymphoid, 39%), which was at extramedullary sites in 18 (47%) patients. The comparison between patients with PDGFRA/PDGFRB vs. FGFR1, JAK2, and ETV6::ABL1 fusion genes revealed a similar occurrence of primary BP (17/104, 16% vs. 8/31 26%, p = 0.32), a lower frequency (5/87, 6% vs. 8/23, 35%, p = 0.003) of and a later progression (median 87 vs. 19 months, p = 0.053) into secondary BP, and a better overall survival from diagnosis of BP (17.1 vs. 1.7 years, p < 0.0008). We conclude that hypereosinophilia with or without monocytosis and various phenotypes of BP occur at variable frequencies in MLN-TK.
1860-1867
Metzgeroth, Georgia
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Steiner, Laurenz
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Naumann, Nicole
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Lübke, Johannes
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Kreil, Sebastian
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Fabarius, Alice
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Haferlach, Claudia
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Haferlach, Torsten
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Hofmann, Wolf-karsten
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Cross, Nicholas C.P.
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Schwaab, Juliana
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Reiter, Andreas
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1 September 2023
Metzgeroth, Georgia
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Steiner, Laurenz
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Naumann, Nicole
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Lübke, Johannes
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Kreil, Sebastian
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Fabarius, Alice
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Haferlach, Claudia
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Haferlach, Torsten
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Hofmann, Wolf-karsten
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Cross, Nicholas C.P.
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Schwaab, Juliana
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Reiter, Andreas
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Metzgeroth, Georgia, Steiner, Laurenz, Naumann, Nicole, Lübke, Johannes, Kreil, Sebastian, Fabarius, Alice, Haferlach, Claudia, Haferlach, Torsten, Hofmann, Wolf-karsten, Cross, Nicholas C.P., Schwaab, Juliana and Reiter, Andreas
(2023)
Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions: reevaluation of the defining characteristics in a registry-based cohort.
Leukemia, 37 (9), .
(doi:10.1038/s41375-023-01958-1).
Abstract
In a registry-based analysis of 135 patients with “myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions” (MLN-TK; FIP1L1::PDGFRA, n = 78; PDGFRB, diverse fusions, n = 26; FGFR1, diverse, n = 9; JAK2, diverse, n = 11; ETV6::ABL1, n = 11), we sought to evaluate the disease-defining characteristics. In 81/135 (60%) evaluable patients, hypereosinophilia (>1.5 × 10 9/l) was observed in 40/44 (91%) FIP1L1::PDGFRA and 7/7 (100%) ETV6::ABL1 positive patients but only in 13/30 (43%) patients with PDGFRB, FGFR1, and JAK2 fusion genes while 9/30 (30%) patients had no eosinophilia. Monocytosis >1 × 10 9/l was identified in 27/81 (33%) patients, most frequently in association with hypereosinophilia (23/27, 85%). Overall, a blast phase (BP) was diagnosed in 38/135 (28%) patients (myeloid, 61%; lymphoid, 39%), which was at extramedullary sites in 18 (47%) patients. The comparison between patients with PDGFRA/PDGFRB vs. FGFR1, JAK2, and ETV6::ABL1 fusion genes revealed a similar occurrence of primary BP (17/104, 16% vs. 8/31 26%, p = 0.32), a lower frequency (5/87, 6% vs. 8/23, 35%, p = 0.003) of and a later progression (median 87 vs. 19 months, p = 0.053) into secondary BP, and a better overall survival from diagnosis of BP (17.1 vs. 1.7 years, p < 0.0008). We conclude that hypereosinophilia with or without monocytosis and various phenotypes of BP occur at variable frequencies in MLN-TK.
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Metzgeroth
- Accepted Manuscript
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s41375-023-01958-1
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Accepted/In Press date: 27 June 2023
e-pub ahead of print date: 15 July 2023
Published date: 1 September 2023
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Funding Information:
This work was supported by the “Deutsche José Carreras Leukämie-Stiftung e.V.” (Grant No. 08 R/2020), Germany. Open Access funding enabled and organized by Projekt DEAL.
Publisher Copyright:
© 2023, The Author(s).
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Local EPrints ID: 480414
URI: http://eprints.soton.ac.uk/id/eprint/480414
ISSN: 0887-6924
PURE UUID: 9ba11be2-cc64-4d5e-a9c9-1f1a125c5774
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Date deposited: 02 Aug 2023 16:30
Last modified: 18 Mar 2024 02:54
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Author:
Georgia Metzgeroth
Author:
Laurenz Steiner
Author:
Nicole Naumann
Author:
Johannes Lübke
Author:
Sebastian Kreil
Author:
Alice Fabarius
Author:
Claudia Haferlach
Author:
Torsten Haferlach
Author:
Wolf-karsten Hofmann
Author:
Juliana Schwaab
Author:
Andreas Reiter
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