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Limited duration of complete remission on ruxolitinib in myeloid neoplasms with PCM1-JAK2 and BCR-JAK2 fusion genes

Limited duration of complete remission on ruxolitinib in myeloid neoplasms with PCM1-JAK2 and BCR-JAK2 fusion genes
Limited duration of complete remission on ruxolitinib in myeloid neoplasms with PCM1-JAK2 and BCR-JAK2 fusion genes
Rearrangements of chromosome band 9p24 are known to be associated with JAK2 fusion genes, e.g., t(8;9)(p22;p24) with a PCM1-JAK2 and t(9;22)(p24;q11) with a BCR-JAK2 fusion gene, respectively. In association with myeloid neoplasms, the clinical course is aggressive, and in absence of effective conventional treatment options, long-term remission is usually only observed after allogeneic stem cell transplantation (ASCT). With the discovery of inhibitors of the JAK2 tyrosine kinase and based on encouraging in vitro and in vivo data, we treated two male patients with myeloid neoplasms and a PCM1-JAK2 or a BCR-JAK2 fusion gene, respectively, with the JAK1/JAK2 inhibitor ruxolitinib. After 12 months of treatment, both patients achieved a complete clinical, hematologic, and cytogenetic response. Non-hematologic toxicity was only grade 1 while no hematologic toxicity was observed. However, remission in both patients was only short-term, with relapse occurring after 18 and 24 months, respectively, making ASCT indispensable in both cases. This data highlight (1) the ongoing importance of cytogenetic analysis for the diagnostic work-up of myeloid neoplasms as it may guide targeted therapy and (2) remission under ruxolitinib may only be short-termed in JAK2 fusion genes but it may be an important bridging therapy prior to ASCT.
233-238
Schwaab, Juliana
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Knut, Marcin
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Haferlach, Claudia
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Metzgeroth, Georgia
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Horny, Hans-Peter
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Chase, Andrew
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Tapper, William
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Score, Joannah
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Waghorn, Katherine
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Naumann, Nicole
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Jawhar, Mohamad
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Fabarius, Alice
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Hofmann, Wolf-Karsten
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Cross, Nicholas C.P.
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Reiter, Andreas
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Schwaab, Juliana
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Knut, Marcin
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Haferlach, Claudia
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Metzgeroth, Georgia
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Horny, Hans-Peter
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Chase, Andrew
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Tapper, William
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Score, Joannah
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Waghorn, Katherine
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Naumann, Nicole
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Jawhar, Mohamad
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Fabarius, Alice
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Hofmann, Wolf-Karsten
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Cross, Nicholas C.P.
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Reiter, Andreas
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Schwaab, Juliana, Knut, Marcin, Haferlach, Claudia, Metzgeroth, Georgia, Horny, Hans-Peter, Chase, Andrew, Tapper, William, Score, Joannah, Waghorn, Katherine, Naumann, Nicole, Jawhar, Mohamad, Fabarius, Alice, Hofmann, Wolf-Karsten, Cross, Nicholas C.P. and Reiter, Andreas (2014) Limited duration of complete remission on ruxolitinib in myeloid neoplasms with PCM1-JAK2 and BCR-JAK2 fusion genes. Annals of Hematology, 94 (2), 233-238. (doi:10.1007/s00277-014-2221-y). (PMID:25260694)

Record type: Article

Abstract

Rearrangements of chromosome band 9p24 are known to be associated with JAK2 fusion genes, e.g., t(8;9)(p22;p24) with a PCM1-JAK2 and t(9;22)(p24;q11) with a BCR-JAK2 fusion gene, respectively. In association with myeloid neoplasms, the clinical course is aggressive, and in absence of effective conventional treatment options, long-term remission is usually only observed after allogeneic stem cell transplantation (ASCT). With the discovery of inhibitors of the JAK2 tyrosine kinase and based on encouraging in vitro and in vivo data, we treated two male patients with myeloid neoplasms and a PCM1-JAK2 or a BCR-JAK2 fusion gene, respectively, with the JAK1/JAK2 inhibitor ruxolitinib. After 12 months of treatment, both patients achieved a complete clinical, hematologic, and cytogenetic response. Non-hematologic toxicity was only grade 1 while no hematologic toxicity was observed. However, remission in both patients was only short-term, with relapse occurring after 18 and 24 months, respectively, making ASCT indispensable in both cases. This data highlight (1) the ongoing importance of cytogenetic analysis for the diagnostic work-up of myeloid neoplasms as it may guide targeted therapy and (2) remission under ruxolitinib may only be short-termed in JAK2 fusion genes but it may be an important bridging therapy prior to ASCT.

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Published date: 27 September 2014
Organisations: Human Development & Health

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Local EPrints ID: 369550
URI: http://eprints.soton.ac.uk/id/eprint/369550
PURE UUID: ce654845-f4ea-4bed-b8ff-4bf4d6747110
ORCID for Andrew Chase: ORCID iD orcid.org/0000-0001-6617-9953
ORCID for William Tapper: ORCID iD orcid.org/0000-0002-5896-1889
ORCID for Nicholas C.P. Cross: ORCID iD orcid.org/0000-0001-5481-2555

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Date deposited: 07 Oct 2014 11:57
Last modified: 15 Mar 2024 03:11

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Contributors

Author: Juliana Schwaab
Author: Marcin Knut
Author: Claudia Haferlach
Author: Georgia Metzgeroth
Author: Hans-Peter Horny
Author: Andrew Chase ORCID iD
Author: William Tapper ORCID iD
Author: Joannah Score
Author: Katherine Waghorn
Author: Nicole Naumann
Author: Mohamad Jawhar
Author: Alice Fabarius
Author: Wolf-Karsten Hofmann
Author: Andreas Reiter

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