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Allogeneic bone marrow transplantation for hypereosinophilic syndrome: long-term follow-up with eradication of FIP1L1-PDGFRA fusion transcript

Bone Marrow Transplantation volume 38, pages 319–320 (2006)Cite this article

As the fusion gene FIP1L1-PDGFRA was identified as being responsible for clonal evolution in some patients clinically diagnosed with hypereosinophilic syndrome (HES), effective treatment with imatinib became available.1 The inhibitory effect of imatinib on the fusion tyrosine kinase leads to molecular remission in a subset of patients after several months of therapy.2 The first report of effective imatinib treatment in HES was published in 2001, but the mechanism of action was not known at that time.3 Here, we describe a patient transplanted for HES in 2001 in whom the FIP1L1-PDGFRA transcript was retrospectively shown to be eradicated after transplantation.

Attempts of allogeneic stem cell transplantation in HES have been published as far back as 1988.4 However, owing to relatively high treatment-related mortality, allogeneic transplantation is reserved only for patients with aggressive disease failing frontline treatment. Introduction of non-myeloablative conditioning has made transplantation available even for patients, with severe organ dysfunction during the course of the disease.5 Papers describing allogeneic transplantation in HES published are mostly case reports or deal with small series of patients. It is therefore difficult to establish a transplantation standard for HES patients, particularly given the heterogeneity of the syndrome.6, 7 Recently, FIP1L1-PDGFRA fusion gene was identified as a detrimental factor causing disease evolution in a subset of patients. Accumulating data confirm imatinib efficacy in those patients. Unfortunately, less than 20% of HES patients carry the mutation.6, 8 Only those patients have a sustained response to imatinib, whereas others mostly show a transient improvement. It is not known whether molecular remissions will be sustained if therapy is interrupted, how long the treatment should be continued or if resistance to imatinib will emerge at some point of treatment. Also, it is not well established whether or not prolonged imatinib administration will result in resolution of organ involvement.2 As in many myeloproliferative disorders, allogeneic stem cell transplantation is the only confirmed curative option in HES. In case of imatinib failure in FIP1L1-PDGFRA-positive patients undergoing transplantation, the fusion transcript may serve as a highly sensitive tool for the evaluation of molecular remission after grafting.

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Authors and Affiliations

  1. Department of Haematology, Medical University of Gdańsk, Gdańsk, Poland

    K Halaburda, W Prejzner, D Szatkowski & A Hellmann

  2. Department of Biology and Genetics, Medical University of Gdańsk, Gdańsk, Poland

    J Limon

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  1. K Halaburda
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  2. W Prejzner
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Correspondence to K Halaburda.

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Halaburda, K., Prejzner, W., Szatkowski, D. et al. Allogeneic bone marrow transplantation for hypereosinophilic syndrome: long-term follow-up with eradication of FIP1L1-PDGFRA fusion transcript. Bone Marrow Transplant 38, 319–320 (2006). https://doi.org/10.1038/sj.bmt.1705437

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