Skip to main content

Advertisement

Log in

Complete molecular remission of chronic eosinophilic leukemia complicated by CNS disease after targeted therapy with imatinib

  • Case Report
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

Many cases of hypereosinophilia, formerly classified as hypereosinophilic syndrome, can now be characterized as chronic eosinophilic leukemia (CEL) based on the demonstration of characteristic genetic markers indicating clonality of hematopoiesis. Here we report on a 33-year-old male patient with central nervous system manifestations of CEL and an excellent response to low-dose imatinib (Glivec). Molecular analysis demonstrated a constitutive activation of the platelet-derived growth factor receptor-alpha (PDGFR-A) as the mechanism of responsiveness to imatinib.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

Abbreviations

BM:

Bone marrow

CEL:

Chronic eosinophilic leukemia

CML:

Chronic myeloid leukemia

CNS:

Central nervous system

HES:

Hypereosinophilic syndrome

FIP1L1:

FIP1-like gene

PB:

Peripheral blood

PDGFR-A/B:

Platelet-derived growth factor receptor-alpha/beta

References

  1. Chusid MJ, Dale DC, West BC, Wolff SM (1975) The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore) 54:1–27

  2. Gleich GJ, Leiferman KM, Pardanani A, Tefferi A, Butterfield JH (2002) Treatment of hypereosinophilic syndrome with imatinib mesilate. Lancet 359:1577–1578

    CAS  PubMed  Google Scholar 

  3. Simon HU, Plotz SG, Dummer R, Blaser K (1999) Abnormal clones of T cells producing interleukin-5 in idiopathic eosinophilia. N Engl J Med 341:1112–1120

    Article  CAS  PubMed  Google Scholar 

  4. Bain BJ (2003) Cytogenetic and molecular genetic aspects of eosinophilic leukaemias. Br J Haematol 122:173–179

    Article  PubMed  Google Scholar 

  5. Bain B, Pierre R, Imbert M, Vardiam JW, Brunning RD, Flandrin G (2001) Chronic eosinophilic leukaemia and the hypereosinophilic syndrome. In: Jaffe E, Harris NL, Stein H, Vardiman JW (eds) World Health classification of tumours: pathology and genetics of tumours of haematopoietic and lymphoid tissues, 1st edn. IARC Press, Lyon, pp 29–31

  6. Apperley JF, Gardembas M, Melo JV, Russell-Jones R, Bain BJ, Baxter EJ, et al. (2002) Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med 347:481–487

    Article  CAS  PubMed  Google Scholar 

  7. Schoch C, Schnittger S, Bursch S, Gerstner D, Hochhaus A, Berger U, et al. (2002) Comparison of chromosome banding analysis, interphase- and hypermetaphase-FISH, qualitative and quantitative PCR for diagnosis and for follow-up in chronic myeloid leukemia: a study on 350 cases. Leukemia 16:53–59

    Article  CAS  PubMed  Google Scholar 

  8. Cross NC, Feng L, Bungey J, Goldman JM (1993) Minimal residual disease after bone marrow transplant for chronic myeloid leukaemia detected by the polymerase chain reaction. Leuk Lymphoma 11 [Suppl 1]:39–43

    Google Scholar 

  9. Cross NC, Melo JV, Feng L, Goldman JM (1994) An optimized multiplex polymerase chain reaction (PCR) for detection of BCR-ABL fusion mRNAs in haematological disorders. Leukemia 8:186–189

    CAS  PubMed  Google Scholar 

  10. Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, et al. (2003) A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 348:1201–1214

    Article  CAS  PubMed  Google Scholar 

  11. Longley BJ Jr, Metcalfe DD, Tharp M, Wang X, Tyrrell L, Lu SZ, et al. (1999) Activating and dominant inactivating c-KIT catalytic domain mutations in distinct clinical forms of human mastocytosis. Proc Natl Acad Sci U S A 96:1609–1614

    Article  CAS  PubMed  Google Scholar 

  12. Goldman JM, Melo JV (2003) Chronic myeloid leukemia—advances in biology and new approaches to treatment. N Engl J Med 349:1451–1464

    Article  CAS  PubMed  Google Scholar 

  13. Cross NC, Reiter A (2002) Tyrosine kinase fusion genes in chronic myeloproliferative diseases. Leukemia 16:1207–1212

    Article  CAS  PubMed  Google Scholar 

  14. Macdonald D, Reiter A, Cross NC (2002) The 8p11 myeloproliferative syndrome: a distinct clinical entity caused by constitutive activation of FGFR1. Acta Haematol 107:101–107

    Article  CAS  PubMed  Google Scholar 

  15. Golub TR, Barker GF, Lovett M, Gilliland DG (1994) Fusion of PDGF receptor beta to a novel ets-like gene, tel, in chronic myelomonocytic leukemia with t(5;12) chromosomal translocation. Cell 77:307–316

    CAS  PubMed  Google Scholar 

  16. Steer EJ, Cross NC (2002) Myeloproliferative disorders with translocations of chromosome 5q31–35: role of the platelet-derived growth factor receptor Beta. Acta Haematol 107:113–122

    Article  CAS  PubMed  Google Scholar 

  17. Cortes J, Ault P, Koller C, Thomas D, Ferrajoli A, Wierda W, et al. (2003) Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome. Blood 101:4714–4716

    Article  CAS  PubMed  Google Scholar 

  18. Coutant G, Bletry O, Prin L, Hauteville D, de Puyfontaine O, Abgrall JF, et al. (1993) Treatment of hypereosinophilic syndromes of myeloproliferative expression with the combination of hydroxyurea and interferon alpha. Apropos of 7 cases. Ann Med Interne (Paris) 144:243–250

    Google Scholar 

  19. Esteva-Lorenzo FJ, Meehan KR, Spitzer TR, Mazumder A (1996) Allogeneic bone marrow transplantation in a patient with hypereosinophilic syndrome. Am J Hematol 51:164–165

    Article  CAS  PubMed  Google Scholar 

  20. Juvonen E, Volin L, Koponen A, Ruutu T (2002) Allogeneic blood stem cell transplantation following non-myeloablative conditioning for hypereosinophilic syndrome. Bone Marrow Transplant 29:457–458

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

We would like to thank PD Dr. Claudia Schoch, Munich and Dr. Schwab, Wiesbaden for performing cytogenetics, FISH, and PCR analysis to exclude classic CML.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Norbert Frickhofen.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Frickhofen, N., Märker-Hermann, E., Reiter, A. et al. Complete molecular remission of chronic eosinophilic leukemia complicated by CNS disease after targeted therapy with imatinib. Ann Hematol 83, 477–480 (2004). https://doi.org/10.1007/s00277-004-0845-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-004-0845-z

Keywords

Navigation