The idiopathic hypereosinophilic syndrome and chronic eosinophilic leukemia

Česká verzia

Authors: L. Chrobák; J. Voglová
Authors place of work: Oddělení klinické hematologie II. interní kliniky Lékařské fakulty UK a FN, Hradec Králové, přednosta prof. MUDr. Jaroslav Malý, CSc.
Published in the journal: Vnitř Lék 2005; 51(12): 1385-1393
Category: Přehledný referát

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Summary

Idiopathic hypereosinophilic syndrome is a heterogenous group of hematological disorders characterized by eosinophilia (> 1.5 x 10⁹/l) persistent for more than 6 months, exclusion of reactive eosinophilia from other causes, such as parasitic infections or allergy, and evidence of end-organ damage [22]. According to World Health Organization the exclusion includes all neoplastic disorders in which eosinophils are part of the neoplastic clone. Excluded should be also T cell population with aberant phenotype and abnormal cytokine production [7], recently considert also as „lymphocytic“ variants of the HES [42]. HES has to be reclassified as chronic eosinophilic leukemia (CEL) when there is evidence for clonality based on the presence of chromosomal abnormalities or inactivation in female patients [7]. The successful empiric treatment of patients with tyrosine kinase inhibitor imatinib (Glivec) suggested the presence of an imatinib-sensitive tyrosine kinase inhibitor. The identification of a specific intersticial chromosoma deletion del(4)(q12;q12) creating the FIP1L1-PDGFRA fusion gene confirmed this hypothesis. Patients carrying this gene should be reclassified as CEL and detection of this gene is a positive predictor for response to imatinib therapy [11,45]. Effective doses of imatinib are 100 mg/day. The side effects are minimal. The only exception is an acute left ventricular dysfunction which has been reported in three patients within the first week of treatment with imatinib [36,38]. Imatinib has been successfully used also in some patients with the constitutively activated thyrosine kinase ETV6-PDGFRβ [1] and in systemic mast cell disease associated with eosinophilia [35]. Other therapeutical options for HES/CEL have been mentioned. The resistence to imatinib and the possibilities how to overcome it are discussed.

Key words:
hypereosinophilia - chronic eosinophilic leukemia - imatinib

Zdroje

1. Apperley JF, Gardebas M, Melo JV et al. Response to imatinib mesylate in patients with chronic myeloproliferative disease with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med 2002; 347: 481-487.

2. Ascione L, DeMichele M, Accadia M et al. Reversal of cardiac abnormalities in a young man with idiopathic hypereosinophilic syndrome using a tyrosine kinase inhibitor. Eur J Echocardiogr 2004; 5: 386-390.

3. Ault P, Cortes J, Koller C et al. Response of idiopathic hypereosinophilic syndrome to treatment with imatinib mesylate. Leuk Res 2002; 26: 881-884.

4. Bain BJ. Eosinophilic leukaemias and the idiopathic hypereosinophilic syndrome. Br J Haematol 1996; 95: 2-9.

5. Bain BJ. Hypereosinophilia. Curr Opin Hematol 2000; 7: 21-25.

6. Bain BJ. Cytogenetic and molecular genetic aspects of eosinophilic leukaemias. Br J Haematol 2003; 122: 173-179.

7. Bain BJ, Pierre R, Imbert M et al. Chronic eosinophilic leukaemia and the hypereosinophilic syndrome. In: Jaffe ES, Harris NL, Stein H et al. World Health Organization classification of tumors: Pathology and genetics of tumours of the haematopoietic and lymphoid tissues. Lyon, France: IARC Press 2001: 1201-1214.

8. Bonaventure C, Mastroianni B, Alessio A et al. Hypereosinophilie paraneoplasique associée à un carcinome hepatocellulaire. Gastroenterol Clin Biol 2003; 27: 1167-1169.

9. Brigden ML. A practical workup for eosinophilia. You can investigate the most likely causes right in your office. Postgrad Med 1999; 105: 207-210.

10. Brito-Babapulle F. The eosinophilias, including the idiopathic hypereosinophilic syndrome. Br J Haematol 2003; 121: 203-223.

11. Cools J, DeAngelo D, Gotlib J et al. 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 2003; 348: 1201-1214.

12. Cools J, Stover EH, Boulton CL et al. PKC412 overcomes resistance to imatinib in a murine model of FIP1L1-PDGFRα-induced myeloproliferative disease. Cancer Cell 2003; 3: 459-469.

13. Cools J, Stover EH, Wlodarska I et al. The FIP1L1-PDGFRα kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia. Curr Opin Hematol 2003; 11: 51-57.

14. Cortes J, Ault P, Koller C et al. Efficacy of imatinib mesylate in treatment of idiopathic hypereosinophilic syndrome Blood 2003; 101: 4714-4716.

15. Cortes J, O´Brien S, Kantarjian H. Discontinuation of imatinib therapy after achieving a molecular response. Blood 2004; 104: 2204-2205.

16. Cooper MA, Akard LP, Thompson JM et al. Hypereosinophilic syndrome: long-term remission following allogeneic stem cell transplant in spite of transient eosinophilic post-transplant. Am J Hematol 2005; 78: 33-36.

17. Garret JK, Jameson SC, Thomson B et al. Anti-interleukin-5 (mepolizumab) therapy for hypereosinophilic syndromes. J Allergy Clin Immunol 2004; 113: 115-119.

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

19. Gudmondsson GS, Ohr J, Leya F et al. An unusual case of recurrent Löffler endomyocarditis of the aortic valve. Arch Pathol Lab Med 2003; 127: 606-609.

20. Gleich GJ, Leiferman KM, Pardanani A et al. Treatment of hypereosinophilic syndrome with imatinib mesylate. Lancet 2002; 359: 1577-1578.

21. Hardy WR, Anderson RE The hypereosinophilic syndromes. Ann Intern Med 1968; 68: 1220-1229.

22. Chusid MJ, Dale DC, West BC et al. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore) 1975; 54: 1-27.

23. Inhorn RC, Aster JC, Roach SA et al. A syndrome of lymphoblastic lymphoma, eosinophilia and myeloid hyperplasia/malignancy associated with t (8;13) (p 11; q 11): description of distinctive clinico-pathologic entity. Blood 1995; 85: 1881-1887.

24. Kim YJ, Prussin C, Martin B et al. Rebound eosinophilia after treatment of hypereosinophilic syndrome and eosinophilic gastroenteritis with monoclonal anti-IL-5 antibody SCH55700. J Allergy Clin Immunol 2004; 114: 1449-1455.

25. Klion AD, Law MA, Noel P et al. Safety and efficacy of the monoclonal anti-interleukin-5 antibody SCH55700 in the treatment of patients with hypereosinophilic syndrome. Blood 2004; 103: 2939-2941.

26. Klion AD, Robyn J, Akin C et al. Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variants of hypereosinophilic syndrome. Blood 2004; 103: 473-478.

27. Krejsek J, Kopecký O. Klinická imunologie. Hradec Králové: Nucleus 2004.

28. Leblond P, Lepers S, Thebaud E et al. Le syndrome d´hypereosinophilie essentielle: à propos d´un cas chez un nourrisson. Arch Pediatr 2004; 11: 219-222.

29. Luppi M, Marasca R, Morselli M et al. Clonal nature of hypereosinophilic syndrome. Blood 1994; 84: 349-350.

30. MacDonald D, Aguiar RCT, Mason PJ et al. A new myeloproliferative disorder associated with chromosomal translocation involving 8p11: A review. Leukemia 1995; 9: 1628-1630.

31. MacDonald D, Reiter A, Cross NCP. The 8p11 myeloproliferative syndrome: a distinct clinical entity caused by constitutive activation of FGFR1. Acta Haematol 2002; 107: 101-107.

32. Maruyoshi H, Nakatani S, Yasumura Y et al. Löffler´s endocarditis associated with unusual ECG change mimicking posterior myocardial infarction. Heart-Vessels 2003; 18: 43-46.

33. Nishie M, Tomiyama M, Kamijo M et al. Acute cholecystitis and duodenitis associated with Churg-Strauss syndrome. Hepatogastroenterology 2003; 50: 998-1002.

34. Orson FM. Intravenous immunoglobulin therapy supresses manifestations of the angioedema with hypereosinophilic syndrome. Am J Med Sci 2003; 326: 94-97.

35. Pardanani A, Elliot M, Li CY et al. Imatinib for systemic mast-cell disease. Lancet 2003; 362: 535-537.

36. Pardanani A, Reeder T, Porrata LF et al. Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders. Blood 2003; 101: 3391-3397.

37. Parrillo JE, Fauci AS, Wolff SM. Therapy of hypereosinophilic syndrome. Ann Intern Med 1978; 89: 167-172.

38. Pitini V, Arrigo C, Azzarello D et al. Serum concentration of cardiac Troponin T in patients with hypereosinophilic syndrome treated with imatinib is predictive of adverse outcomes. Blood 2003; 102: 3456-3457.

39. Pitini V, Teti D, Arrigo C et al. Alemtuzumab therapy for idiopathic hypereosinophilic syndrome with abnormal T cells: a case report. Br J Haematol 2004; 127: 474.

40. Plötz SG, Simon HU, Darson V et al. Use of an anti-interleukin-5 antibody in the hypereosinophilic syndrome with eosinophilic dermatitis. N Engl J Med 2003; 349: 2334-2339.

41. Rosenberg ME. Mechanisms of disease: eosinophilia. N Engl J Med 1985; 313: 1485-1492.

42. Roufosse F, Cogan E, Goldman M. The hypereosinophilic syndrome revisited. Annu Rev Med 2003; 54: 169-184.

43. Sefcick A, Sowter D, DasGusta E et al. Alemtuzumab therapy for refractory idiopathic hypereosinophilic syndrome. Br J Haematol 2004; 124: 558-559.

44. Schaller JL, Burkland GA. Rapid and complete control of idiopathic hypereosinophilia with imatinib mesylate. Med Gen Med 2001; 3: 9.

45. Steer EJ, Cross NCP. Myeloproliferative disorders with translocations of chromosome 5q 31-35; role of the platelet-derived growth factor receptor beta. Acta Haematol 2002; 107: 113-122.

46. Tefferi A, Pardanani A. Imatinib treatment-induced cardiomyopathy in hypereosinophilic syndrome. Blood 2003; 102: 3457.

47. Vandenberghe P, Wlodarska J, Michaux L et al. Clinical and molecular features of FIP1L1-PDGFRA(+) chronic eosinophilic leukemia. Leukemia 2004; 18: 734-742.

48. Weller PF, Bubley G. The idiopathic hypereosinophilic syndrome. Blood 1994; 83: 2759-2779.

49. Zittoun J, Farcet JP, Marquet J et al. Cobalamin (vitamin B12) and B12 binding proteins in hypereosinophilic syndromes and secondary eosinophilia. Blood 1984; 63: 779-783.