Journal Mobile Options
Table of Contents
Vol. 114, No. 1, 2005
Issue release date: June 2005
Acta Haematol 2005;114:52–60
(DOI:10.1159/000085562)

Modern Diagnosis and Treatment of Primary Eosinophilia

Tefferi A.
Divisions of Hematology and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, Minn., USA

Individual Users: Register with Karger Login Information

Please create your User ID & Password





Contact Information











I have read the Karger Terms and Conditions and agree.

To view the fulltext, please log in

To view the pdf, please log in

Abstract

The recent discovery of an eosinophilia-specific, imatinib-sensitive, karyotypically occult but fluorescence in situ hybridization-apparent molecular lesion in a subset of patients with blood eosinophilia has transformed the diagnostic as well as treatment approach to eosinophilic disorders. Primary (i.e. nonreactive) eosinophilia is considered either ‘clonal’ or ‘idiopathic’ based on the presence or absence, respectively, of either a molecular or bone marrow histological evidence for a myeloid neoplasm. Clonal eosinophilia might accompany a spectrum of clinicopathological entities, the minority of whom are molecularly characterized; Fip1-like-1-platelet-derived growth factor receptor α (FIP1L1-PDGFRA+) systemic mastocytosis, platelet-derived growth factor receptor β (PDGFRB)-rearranged atypical myeloproliferative disorder, chronic myeloid leukemia, and the 8p11 syndrome that is associated with fibroblast growth factor receptor 1 (FGFR1) rearrangement. Hypereosinophilic syndrome (HES) is a subcategory of idiopathic eosinophilia and is characterized by an absolute eosinophil count of ≧1.5 × 109/l for at least 6 months as well as eosinophil-mediated tissue damage. At present, a working diagnosis of primary eosinophilia mandates a bone marrow examination, karyotype analysis, and additional molecular studies in order to provide the patient with accurate prognostic information as well as select appropriate therapy. For example, the presence of either PDGFRA or PDGFRB mutations warrants the use of imatinib in clonal eosinophilia. In HES, prednisone, hydroxyurea, and interferon-α constitute first-line therapy, whereas imatinib, cladribine, and monoclonal antibodies to either interleukin-5 (mepolizumab) or CD52 (alemtuzumab) are considered investigational. Allogeneic transplantation offers a viable treatment option for drug-refractory cases.



Copyright / Drug Dosage

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

References

  1. Brito-Babapulle F: The eosinophilias, including the idiopathic hypereosinophilic syndrome. Br J Haematol 2003;121:203–223.
  2. Blatt PM, Rothstein G, Miller HL, Cathey WJ: Loffler’s endomyocardial fibrosis with eosinophilia in association with acute lymphoblastic leukemia. Blood 1974;44:489–493.
  3. Sanada I, Asou N, Kojima S, Kawano F, Shido T, Takatsuki K: Acute myelogenous leukemia (FAB M1) associated with t(5;16) and eosinophilia. Report of an additional case. Cancer Genet Cytogenet 1989;43:139–141.
  4. Kuroda J, Kimura S, Akaogi T, Hayashi H, Yamano T, Sasai Y, Horiike S, Taniwaki M, Abe T, Kobayashi Y, Kondo M: Myelodysplastic syndrome with clonal eosinophilia accompanied by eosinophilic pulmonary interstitial infiltration. Acta Haematol 2000;104:119–123.
  5. Keung YK, Beaty M, Steward W, Jackle B, Pettnati M: Chronic myelocytic leukemia with eosinophilia, t(9;12)(q34;p13), and ETV6-ABL gene rearrangement: Case report and review of the literature. Cancer Genet Cytogenet 2002;138:139–142.
  6. Wilkinson K, Velloso ER, Lopes LF, Lee C, Aster JC, Shipp MA, Aguiar RC: Cloning of the t(1;5)(q23;q33) in a myeloproliferative disorder associated with eosinophilia: Involvement of PDGFRB and response to imatinib. Blood 2003;102:4187–4190.
  7. Bain BJ: Cytogenetic and molecular genetic aspects of eosinophilic leukaemias. Br J Haematol 2003;122:173–179.
  8. Yam LT, Yam CF, Li CY: Eosinophilia in systemic mastocytosis. Am J Clin Pathol 1980;73:48–54.
  9. Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick H, Sultan C, Cox C: The chronic myeloid leukaemias: Guidelines for distinguishing chronic granulocytic, atypical chronic myeloid, and chronic myelomonocytic leukaemia. Proposals by the French-American-British Cooperative Leukaemia Group. Br J Haematol 1994;87:746–754.
  10. Ma SK, Kwong YL, Shek TW, Wan TS, Chow EY, Chan JC, Chan LC: The role of trisomy 8 in the pathogenesis of chronic eosinophilic leukemia. Hum Pathol 1999;30:864–868.
  11. Bain BJ: Relationship between idiopathic hypereosinophilic syndrome, eosinophilic leukemia, and systemic mastocytosis. Am J Hematol 2004;77:82–85.
  12. Pardanani A, Brockman SR, Paternoster SF, Flynn HC, Ketterling RP, Lasho TL, Ho CL, Li CY, Dewald GW, Tefferi A: FIP1L1-PDGFRA fusion: Prevalence and clinicopathologic correlates in 89 consecutive patients with moderate to severe eosinophilia. Blood 2004;104:3038–3045.
  13. Steer EJ, Cross NC: Myeloproliferative disorders with translocations of chromosome 5q31–35: Role of the platelet-derived growth factor receptor beta. Acta Haematol 2002;107:113–122.
  14. Macdonald D, Reiter A, Cross NC: The 8p11 myeloproliferative syndrome: A distinct clinical entity caused by constitutive activation of FGFR1. Acta Haematol 2002;107:101–107.
  15. Chusid MJ, Dale DC, West BC, Wolff SM: The hypereosinophilic syndrome: Analysis of fourteen cases with review of the literature. Medicine (Baltimore) 1975;54:1–27.
  16. Smith KJ, Jacobson E, Hamza S, Skelton H: Unexplained hypereosinophilia and the need for cytogenetic and molecular genetic analyses. Arch Dermatol 2004;140:584–588.
  17. Bigoni R, Cuneo A, Roberti MG, Milani R, Bardi A, Cavazzini F, Minotto C, Castoldi G: Cytogenetic and molecular cytogenetic characterization of 6 new cases of idiopathic hypereosinophilic syndrome. Haematologica 2000;85:486–491.
  18. Brown NJ, Stein RS: Idiopathic hypereosinophilic syndrome progressing to acute myelomonocytic leukemia with chloromas. South Med J 1989;82:1303–1305.
  19. Owen J, Scott JG: Transition of the hypereosinophilic syndrome to myelomonocytic leukemia. Can Med Assoc J 1979;121:1489–1491.
  20. Yoo TJ, Orman SV, Patil SR, Dorminey C, Needleman S, Rajtora D, Graves N, Ackerman L, Taylor WW: Evolution to eosinophilic leukemia with a t(5:11) translocation in a patient with idiopathic hypereosinophilic syndrome. Cancer Genet Cytogenet 1984;11:389–384.
  21. Needleman SW, Mane SM, Gutheil JC, Kapil V, Heyman MR, Testa JR: Hypereosinophilic syndrome with evolution to myeloproliferative disorder: Temporal relationship to loss of Y chromosome and c-N-ras activation. Hematol Pathol 1990;4:149–155.
  22. Chang HW, Leong KH, Koh DR, Lee SH: Clonality of isolated eosinophils in the hypereosinophilic syndrome. Blood 1999;93:1651–1657.
  23. Malcovati L, La Starza R, Merante S, Pietra D, Mecucci C, Cazzola M: Hypereosinophilic syndrome and cyclic oscillations in blood cell counts. A clonal disorder of hematopoiesis originating in a pluripotent stem cell. Haematologica 2004;89:497–499.
  24. Roufosse F, Schandene L, Sibille C, Willard-Gallo K, Kennes B, Efira A, Goldman M, Cogan E: Clonal Th2 lymphocytes in patients with the idiopathic hypereosinophilic syndrome. Br J Haematol 2000;109:540–548.
  25. Raghavachar A, Fleischer S, Frickhofen N, Heimpel H, Fleischer B: T lymphocyte control of human eosinophilic granulopoiesis. Clonal analysis in an idiopathic hypereosinophilic syndrome. J Immunol 1987;139:3753–3758.
  26. Means-Markwell M, Burgess T, deKeratry D, O’Neil K, Mascola J, Fleisher T, Lucey D: Eosinophilia with aberrant T cells and elevated serum levels of interleukin-2 and interleukin-15. N Engl J Med 2000;342:1568–1571.
  27. Kobayashi S, Inokuma S, Setoguchi K, Kono H, Abe K: Incidence of peripheral blood eosinophilia and the threshold eosinophile count for indicating hypereosinophilia-associated diseases. Allergy 2002;57:950–956.
  28. Drage LA, Davis MD, De Castro F, Van Keulen V, Weiss EA, Gleich GJ, Leiferman KM: Evidence for pathogenic involvement of eosinophils and neutrophils in Churg-Strauss syndrome. J Am Acad Dermatol 2002;47:209–216.
  29. Apperley JF, Gardembas M, Melo JV, Russell-Jones R, Bain BJ, Baxter EJ, Chase A, Chessells JM, Colombat M, Dearden CE, Dimitrijevic S, Mahon FX, Marin D, Nikolova Z, Olavarria E, Silberman S, Schultheis B, Cross NC, Goldman JM: Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med 2002;347:481–487.
  30. Druker BJ, Talpaz M, Resta DJ, Peng B, Buchdunger E, Ford JM, Lydon NB, Kantarjian H, Capdeville R, Ohno-Jones S, Sawyers CL: Efficacy and safety of a specific inhibitor of the BCR-ABL tyrosine kinase in chronic myeloid leukemia. N Engl J Med 2001;344:1031–1037.
  31. Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, Kutok J, Clark J, Galinsky I, Griffin JD, Cross NC, Tefferi A, Malone J, Alam R, Schrier SL, Schmid J, Rose M, Vandenberghe P, Verhoef G, Boogaerts M, Wlodarska I, Kantarjian H, Marynen P, Coutre SE, Stone R, Gilliland DG: 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.
  32. Pardanani A, Ketterling RP, Brockman SR, Flynn HC, Paternoster SF, Shearer BM, Reeder TL, Li CY, Cross NCP, Cools J, Gilliland DG, Dewald GW, Tefferi A: CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib therapy. Blood 2003;102:3093–3096.
  33. Elliott MA, Pardanani A, Li CY, Tefferi A: Immunophenotypic normalization of aberrant mast cells accompanies histological remission in imatinib-treated patients with eosinophilia-associated mastocytosis. Leukemia 2004;18:1027–1029.
  34. Klion AD, Noel P, Akin C, Law MA, Gilliland DG, Cools J, Metcalfe DD, Nutman TB: Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness. Blood 2003;101:4660–4666.
  35. Harley JB, Fauci AS, Gralnick HR: Noncardiovascular findings associated with heart disease in the idiopathic hypereosinophilic syndrome. Am J Cardiol 1983;52:321–324.
  36. Gleich GJ, Schroeter AL, Marcoux JP, Sachs MI, O’Connell EJ, Kohler PF: Episodic angioedema associated with eosinophilia. N Engl J Med 1984;310:1621–1626.
  37. Kim Y, Lee KS, Choi DC, Primack SL, Im JG: The spectrum of eosinophilic lung disease: Radiologic findings. J Comput Assist Tomogr 1997;21:920–930.
  38. Kang EY, Shim JJ, Kim JS, Kim KI: Pulmonary involvement of idiopathic hypereosinophilic syndrome: CT findings in five patients. J Comput Assist Tomogr 1997;21:612–615.
  39. Garrett JK, Jameson SC, Thomson B, Collins MH, Wagoner LE, Freese DK, Beck LA, Boyce JA, Filipovich AH, Villanueva JM, Sutton SA, Assa’ad AH, Rothenberg ME: Anti-interleukin-5 (mepolizumab) therapy for hypereosinophilic syndromes. J Allergy Clin Immunol 2004;113:115–119.
  40. Sato Y, Taniguchi R, Yamada T, Nagai K, Makiyama T, Okada H, Matsumori A, Sasayama S, Takatsu Y: Measurement of serum concentrations of cardiac troponin T in patients with hypereosinophilic syndrome: A sensitive non-invasive marker of cardiac disorder. Intern Med 2000;39:350.
  41. Pitini V, Arrigo C, Azzarello D, La Gattuta G, Amata C, Righi M, Coglitore S: Serum concentration of cardiac Troponin T in patients with hypereosinophilic syndrome treated with imatinib is predictive of adverse outcomes. Blood 2003;102:3456–3457; author reply 3457.
  42. Ommen SR, Seward JB, Tajik AJ: Clinical and echocardiographic features of hypereosinophilic syndromes. Am J Cardiol 2000;86:110–113.
  43. Corradi D, Vaglio A, Maestri R, Legname V, Leonardi G, Bartoloni G, Buzio C: Eosinophilic myocarditis in a patient with idiopathic hypereosinophilic syndrome: Insights into mechanisms of myocardial cell death. Hum Pathol 2004;35:1160–1163.
  44. Tai PC, Ackerman SJ, Spry CJ, Dunnette S, Olsen EG, Gleich GJ: Deposits of eosinophil granule proteins in cardiac tissues of patients with eosinophilic endomyocardial disease. Lancet 1987;1:643–647.
  45. Savage DG, Antman KH: Imatinib mesylate – A new oral targeted therapy. N Engl J Med 2002;346:683–693.
  46. Gilliland G, Cools J, Stover EH, Wlodarska I, Marynen P: FIP1L1-PDGFRalpha in hypereosinophilic syndrome and mastocytosis. Hematol J 2004;5(suppl 3):S133–S137.

    External Resources

  47. Vandenberghe P, Wlodarska I, Michaux L, Zachee P, Boogaerts M, Vanstraelen D, Herregods MC, Van Hoof A, Selleslag D, Roufosse F, Maerevoet M, Verhoef G, Cools J, Gilliland DG, Hagemeijer A, Marynen P: Clinical and molecular features of FIP1L1-PDFGRA (+) chronic eosinophilic leukemias. Leukemia 2004;18:734–742.
  48. Klion AD, Robyn J, Akin C, Noel P, Brown M, Law M, Metcalfe DD, Dunbar C, Nutman TB: Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variant of hypereosinophilic syndrome. Blood 2004;103:473–478.
  49. Martinelli G, Malagola M, Ottaviani E, Rosti G, Trabacchi E, Baccarani M: Imatinib mesylate can induce complete molecular remission in FIP1L1-PDGFR-α positive idiopathic hypereosinophilic syndrome. Haematologica 2004;89:236–237.
  50. Frickhofen N, Marker-Hermann E, Reiter A, Walz C, Jung B, Bauer H, Hochhaus A: Complete molecular remission of chronic eosinophilic leukemia complicated by CNS disease after targeted therapy with imatinib. Ann Hematol 2004;83:477–480.
  51. Rose C, Dupire S, Roche-Lestienne C, Grardel N, Bourgeois E, Cambier N, Preudhomme C: Sustained molecular response with imatinib in a leukemic form of idiopathic hypereosinophilic syndrome in relapse after allograft. Leukemia 2004;18:354–355.
  52. Trempat P, Villalva C, Laurent G, Armstrong F, Delsol G, Dastugue N, Brousset P: Chronic myeloproliferative disorders with rearrangement of the platelet-derived growth factor alpha receptor: A new clinical target for STI571/Glivec. Oncogene 2003;22:5702–5706.
  53. Safley AM, Sebastian S, Collins TS, Tirado CA, Stenzel TT, Gong JZ, Goodman BK: Molecular and cytogenetic characterization of a novel translocation t(4;22) involving the breakpoint cluster region and platelet-derived growth factor receptor-alpha genes in a patient with atypical chronic myeloid leukemia. Genes Chromosomes Cancer 2004;40:44–50.
  54. Koury MJ, Newman JH, Murray JJ: Reversal of hypereosinophilic syndrome and lymphomatoid papulosis with mepolizumab and imatinib. Am J Med 2003;115:587–589.
  55. Ascione L, De Michele M, Accadia M, Spadaro P, Rumolo S, Tuccillo B: Reversal of cardiac abnormalities in a young man with idiopathic hypereosinophilic syndrome using a tyrosine kinase inhibitor. Eur J Echocardiogr 2004;5:386–390.
  56. Schoffski P, Ganser A, Pascheberg U, Busche G, Gaede B, Hertenstein B: Complete haematological and cytogenetic response to interferon alpha-2a of a myeloproliferative disorder with eosinophilia associated with a unique t(4;7) aberration. Ann Hematol 2000;79:95–98.
  57. Pardanani A, Reeder T, Porrata LF, Li CY, Tazelaar HD, Baxter EJ, Witzig TE, Cross NC, Tefferi A: Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders. Blood 2003;101:3391–3397.
  58. Seshadri T, Seymour JF, McArthur GA: Oligospermia in a patient receiving imatinib therapy for the hypereosinophilic syndrome. N Engl J Med 2004;351:2134–2135.
  59. Pitini V, Arrigo C, Teti D, Barresi G, Righi M, Alo G: Response to STI571 in chronic myelomonocytic leukemia with platelet derived growth factor beta receptor involvement: A new case report. Haematologica 2003;88:ECR18.
  60. Magnusson MK, Meade KE, Nakamura R, Barrett J, Dunbar CE: Activity of STI571 in chronic myelomonocytic leukemia with a platelet-derived growth factor beta receptor fusion oncogene. Blood 2002;100:1088–1091.
  61. Wittman B, Horan J, Baxter J, Goldberg J, Felgar R, Baylor E, Cromwell B, Cross N, Bennett JM: A 2-year-old with atypical CML with a t(5;12)(q33;p13) treated successfully with imatinib mesylate. Leuk Res 2004;28(suppl 1):65–69.

    External Resources

  62. Golub TR, Barker GF, Lovett M, Gilliland DG: Fusion of PDGF receptor beta to a novel ets-like gene, tel, in chronic myelomonocytic leukemia with t(5;12) chromosomal translocation. Cell 1994;77:307–316.
  63. Baxter EJ, Kulkarni S, Vizmanos JL, Jaju R, Martinelli G, Testoni N, Hughes G, Salamanchuk Z, Calasanz MJ, Lahortiga I, Pocock CF, Dang R, Fidler C, Wainscoat JS, Boultwood J, Cross NC: Novel translocations that disrupt the platelet-derived growth factor receptor beta (PDGFRB) gene in BCR-ABL-negative chronic myeloproliferative disorders. Br J Haematol 2003;120:251–256.
  64. Granjo E, Lima M, Lopes JM, Doria S, Orfao A, Ying S, Barata LT, Miranda M, Cross NC, Bain BJ: Chronic eosinophilic leukaemia presenting with erythroderma, mild eosinophilia and hyper-IgE: Clinical, immunological and cytogenetic features and therapeutic approach. A case report. Acta Haematol 2002;107:108–112.
  65. Gupta R, Knight CL, Bain BJ: Receptor tyrosine kinase mutations in myeloid neoplasms. Br J Haematol 2002;117:489–508.
  66. Grand FH, Burgstaller S, Kuhr T, Baxter EJ, Webersinke G, Thaler J, Chase AJ, Cross NC: p53-Binding protein 1 is fused to the platelet-derived growth factor receptor beta in a patient with a t(5;15)(q33;q22) and an imatinib-responsive eosinophilic myeloproliferative disorder. Cancer Res 2004;64:7216–7219.
  67. Vizmanos JL, Novo FJ, Roman JP, Baxter EJ, Lahortiga I, Larrayoz MJ, Odero MD, Giraldo P, Calasanz MJ, Cross NC: NIN, a gene encoding a CEP110-like centrosomal protein, is fused to PDGFRB in a patient with a t(5;14)(q33;q24) and an imatinib-responsive myeloproliferative disorder. Cancer Res 2004;64:2673–2676.
  68. Abe A, Emi N, Tanimoto M, Terasaki H, Marunouchi T, Saito H: Fusion of the platelet-derived growth factor receptor beta to a novel gene CEV14 in acute myelogenous leukemia after clonal evolution. Blood 1997;90:4271–4277.
  69. Luciano L, Catalano L, Sarrantonio C, Guerriero A, Califano C, Rotoli B: AlphaIFN-induced hematologic and cytogenetic remission in chronic eosinophilic leukemia with t(1;5). Haematologica 1999;84:651–653.
  70. Malbrain ML, Van den Bergh H, Zachee P: Further evidence for the clonal nature of the idiopathic hypereosinophilic syndrome: Complete haematological and cytogenetic remission induced by interferon-alpha in a case with a unique chromosomal abnormality. Br J Haematol 1996;92:176–183.
  71. Yamada O, Kitahara K, Imamura K, Ozasa H, Okada M, Mizoguchi H: Clinical and cytogenetic remission induced by interferon-alpha in a patient with chronic eosinophilic leukemia associated with a unique t(3;9;5) translocation. Am J Hematol 1998;58:137–141.
  72. Chen J, DeAngelo DJ, Kutok JL, Williams IR, Lee BH, Wadleigh M, Duclos N, Cohen S, Adelsperger J, Okabe R, Coburn A, Galinsky I, Huntly B, Cohen PS, Meyer T, Fabbro D, Roesel J, Banerji L, Griffin JD, Xiao S, Fletcher JA, Stone RM, Gilliland DG: PKC412 inhibits the zinc finger 198-fibroblast growth factor receptor 1 fusion tyrosine kinase and is active in treatment of stem cell myeloproliferative disorder. Proc Natl Acad Sci USA 2004;101:14479–14484.
  73. Pardanani A, Elliott M, Reeder T, Li C-Y, Baxter EJ, Cross NCP, Tefferi A: Imatinib for systemic mast-cell disease. Lancet 2003;362:535–536.
  74. Quiquandon I, Claisse JF, Capiod JC, Delobel J, Prin L: alpha-Interferon and hypereosinophilic syndrome with trisomy 8: Karyotypic remission. Blood 1995;85:2284–2285.
  75. Butterfield JH: Diverse clinical outcomes of eosinophilic patients with T-cell receptor gene rearrangements: The emerging diagnostic importance of molecular genetics testing. Am J Hematol 2001;68:81–86.
  76. Gleich GJ, Adolphson CR, Leiferman KM: The biology of the eosinophilic leukocyte. Annu Rev Med 1993;44:85–101.
  77. Hamann KJ, Gleich GJ, Checkel JL, Loegering DA, McCall JW, Barker RL: In vitro killing of microfilariae of Brugia pahangi and Brugia malayi by eosinophil granule proteins. J Immunol 1990;144:3166–3173.
  78. Lehrer RI, Szklarek D, Barton A, Ganz T, Hamann KJ, Gleich GJ: Antibacterial properties of eosinophil major basic protein and eosinophil cationic protein. J Immunol 1989;142:4428–4434.
  79. Torpier G, Colombel JF, Mathieu-Chandelier C, Capron M, Dessaint JP, Cortot A, Paris JC, Capron A: Eosinophilic gastroenteritis: Ultrastructural evidence for a selective release of eosinophil major basic protein. Clin Exp Immunol 1988;74:404–408.
  80. Slifman NR, Loegering DA, McKean DJ, Gleich GJ: Ribonuclease activity associated with human eosinophil-derived neurotoxin and eosinophil cationic protein. J Immunol 1986;137:2913–2917.
  81. Young JD, Peterson CG, Venge P, Cohn ZA: Mechanism of membrane damage mediated by human eosinophil cationic protein. Nature 1986;321:613–616.
  82. Sorrentino S, Glitz DG, Hamann KJ, Loegering DA, Checkel JL, Gleich GJ: Eosinophil-derived neurotoxin and human liver ribonuclease. Identity of structure and linkage of neurotoxicity to nuclease activity. J Biol Chem 1992;267:14859–14865.
  83. Sunohara N, Furukawa S, Nishio T, Mukoyama M, Satoyoshi E: Neurotoxicity of human eosinophils towards peripheral nerves. J Neurol Sci 1989;92:1–7.
  84. Chen KR, Su WP, Pittelkow MR, Conn DL, George T, Leiferman KM: Eosinophilic vasculitis in connective tissue disease. J Am Acad Dermatol 1996;35:173–182.
  85. Shah AM, Brutsaert DL, Meulemans AL, Andries LJ, Capron M: Eosinophils from hypereosinophilic patients damage endocardium of isolated feline heart muscle preparations. Circulation 1990;81:1081–1088.
  86. Spry CJ, Davies J, Tai PC, Olsen EG, Oakley CM, Goodwin JF: Clinical features of fifteen patients with the hypereosinophilic syndrome. Q J Med 1983;52:1–22.
  87. Elouaer-Blanc L, Zafrani ES, Farcet JP, Saint-Marc Girardin MF, Mathieu D, Dhumeaux D: Hepatic vein obstruction in idiopathic hypereosinophilic syndrome. Arch Intern Med 1985;145:751–753.
  88. Vargas CA, Maldonado O, Botero RC, Jeffers LJ, Parker T, Reddy KR, Schiff ER: Budd-Chiari syndrome associated with the hypereosinophilic syndrome. Am J Gastroenterol 1993;88:1802–1803.
  89. Parrillo JE, Fauci AS, Wolff SM: Therapy of the hypereosinophilic syndrome. Ann Intern Med 1978;89:167–172.
  90. Schooley RT, Flaum MA, Gralnick HR, Fauci AS: A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations. Blood 1981;58:1021–1026.
  91. Murphy PT, Fennelly DF, Stuart M, O’Donnell JR: Alfa-interferon in a case of hypereosinophilic syndrome. Br J Haematol 1990;75:619–620.
  92. Fruehauf S, Fiehn C, Haas R, Doehner H, Hunstein W: Sustained remission of idiopathic hypereosinophilic syndrome following alpha-interferon therapy. Acta Haematol 1993;89:91–93.
  93. Butterfield JH, Gleich GJ: Response of six patients with idiopathic hypereosinophilic syndrome to interferon alfa. J Allergy Clin Immunol 1994;94:1318–1326.
  94. Ceretelli S, Capochiani E, Petrini M: Interferon-alpha in the idiopathic hypereosinophilic syndrome: Consideration of five cases. Ann Hematol 1998;77:161–164.
  95. Acaba L, Mangual A, Velez Garcia E: Excellent response to interferon therapy in a patient with hypereosinophilic syndrome and elevated serum immunoglobulin E levels. Bol Asoc Med PR 2000;92:59–62.
  96. Zielinski RM, Lawrence WD: Interferon-alpha for the hypereosinophilic syndrome. Ann Intern Med 1990;113:716–718.
  97. van den Anker-Lugtenburg PJ, van ’t Veer MB: Alpha-interferon in a case of hypereosinophilic syndrome. Br J Haematol 1991;77:258.
  98. Busch FW, Schmidt H, Steinke B: Alpha-interferon for the hypereosinophilic syndrome. Ann Intern Med 1991;114:338–339.
  99. Bockenstedt PL, Santinga JT, Bolling SF: alpha-Interferon treatment for idiopathic hypereosinophilic syndrome. Am J Hematol 1994;45:248–251.
  100. Butterfield JH, Gleich GJ: Interferon-alpha treatment of six patients with the idiopathic hypereosinophilic syndrome. Ann Intern Med 1994;121:648–653.
  101. Canonica GW, Passalacqua G, Pronzato C, Corbetta L, Bagnasco M: Effective long-term alpha-interferon treatment for hypereosinophilic syndrome. J Allergy Clin Immunol 1995;96:131–133.
  102. Papo T, Piette JC, Hermine O: Treatment of the hypereosinophilic syndrome with interferon-alpha. Ann Intern Med 1995;123:155–156.
  103. Yoon TY, Ahn GB, Chang SH: Complete remission of hypereosinophilic syndrome after interferon-alpha therapy: Report of a case and literature review. J Dermatol 2000;27:110–115.
  104. Baratta L, Afeltra A, Delfino M, De Castro S, Giorgino F, Rossi-Fanelli F: Favorable response to high-dose interferon-alpha in idiopathic hypereosinophilic syndrome with restrictive cardiomyopathy – Case report and literature review. Angiology 2002;53:465–470.
  105. Demiroglu H, Dundar S: Combination of interferon-alpha and hydroxyurea in the treatment of idiopathic hypereosinophilic syndrome. Br J Haematol 1997;97:928–930.
  106. Schandene L, Roufosse F, de Lavareille A, Stordeur P, Efira A, Kennes B, Cogan E, Goldman M: Interferon alpha prevents spontaneous apoptosis of clonal Th2 cells associated with chronic hypereosinophilia. Blood 2000;96:4285–4292.
  107. Aldebert D, Lamkhioued B, Desaint C, Gounni AS, Goldman M, Capron A, Prin L, Capron M: Eosinophils express a functional receptor for interferon alpha: Inhibitory role of interferon alpha on the release of mediators. Blood 1996;87:2354–2360.
  108. Zabel P, Schlaak M: Cyclosporin for hypereosinophilic syndrome. Ann Hematol 1991;62:230–231.
  109. Nadarajah S, Krafchik B, Roifman C, Horgan-Bell C: Treatment of hypereosinophilic syndrome in a child using cyclosporine: Implication for a primary T-cell abnormality. Pediatrics 1997;99:630–633.
  110. Sakamoto K, Erdreich-Epstein A, deClerck Y, Coates T: Prolonged clinical response to vincristine treatment in two patients with idiopathic hypereosinophilic syndrome. Am J Pediatr Hematol Oncol 1992;14:348–351.
  111. Marshall GM, White L: Effective therapy for a severe case of the idiopathic hypereosinophilic syndrome. Am J Pediatr Hematol Oncol 1989;11:178–183.
  112. Cofrancesco E, Cortellaro M, Pogliani E, Boschetti C, Salvatore M, Polli EE: Response to vincristine treatment in a case of idiopathic hypereosinophilic syndrome with multiple clinical manifestations. Acta Haematol 1984;72:21–25.
  113. Ueno NT, Zhao S, Robertson LE, Consoli U, Andreeff M: 2-Chlorodeoxyadenosine therapy for idiopathic hypereosinophilic syndrome. Leukemia 1997;11:1386–1390.
  114. Smit AJ, van Essen LH, de Vries EG: Successful long-term control of idiopathic hypereosinophilic syndrome with etoposide. Cancer 1991;67:2826–2827.
  115. Schaller JL, Burkland GA: Case report: Rapid and complete control of idiopathic hypereosinophilia with imatinib mesylate. MedGenMed 2001;3:9.
  116. Gleich GJ, Leiferman KM, Pardanani A, Tefferi A, Butterfield JH: Treatment of hypereosinophilic syndrome with imatinib mesylate. Lancet 2002;359:1577–1578.
  117. Ault P, Cortes J, Koller C, Kaled ES, Kantarjian H: Response of idiopathic hypereosinophilic syndrome to treatment with imatinib mesylate. Leuk Res 2002;26:881–884.
  118. Cortes J, Ault P, Koller C, Thomas D, Ferrajoli A, Wierda W, Rios MB, Letvak L, Kaled ES, Kantarjian H: Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome. Blood 2003;101:4714–4716.
  119. Pottier P, Planchon B, Grossi O: [Complete remission with imatinib mesylate (Glivec) of an idiopathic hypereosinophilic syndrome associated with a cutaneous mastocytosis after failure of interferon-alpha]. Rev Med Interne 2003;24:542–546.
  120. Salem Z, Zalloua PA, Chehal A, Bitar N, Abboud M, Kadri A, Chami B, Bazarbachi A: Effective treatment of hypereosinophilic syndrome with imatinib mesylate. Hematol J 2003;4:410–412.
  121. Musto P, Falcone A, Sanpaolo G, Bodenizza C, Perla G, Minervini MM, Cascavilla N, Dell’Olio M, La Sala A, Mantuano S, Melillo L, Nobile M, Scalzulli PR, Bisceglia M, Carella AM: Heterogeneity of response to imatinib-mesylate (glivec) in patients with hypereosinophilic syndrome: Implications for dosing and pathogenesis. Leuk Lymphoma 2004;45:1219–1222.
  122. Tan D, Hwang W, Ng HJ, Goh YT, Tan P: Successful treatment of idiopathic hypereosinophilic syndrome with imatinib mesylate: A case report. Int J Hematol 2004;80:75–77.
  123. Payne SM, Kovacs MJ: Imatinib mesylate treatment in two patients with idiopathic hypereosinophilic syndrome. Ann Pharmacother 2004;38:1215–1218.
  124. Wolf D, Gastl G, Rumpold H: [Complete remission of an idiopathic hypereosinophilic syndrome while using imatinib]. Dtsch Med Wochenschr 2004;129:2104–2106.
  125. Plotz SG, Simon HU, Darsow U, Simon D, Vassina E, Yousefi S, Hein R, Smith T, Behrendt H, Ring J: Use of an anti-interleukin-5 antibody in the hypereosinophilic syndrome with eosinophilic dermatitis. N Engl J Med 2003;349:2334–2339.
  126. Kim YJ, Prussin C, Martin B, Law MA, Haverty TP, Nutman TB, Klion AD: 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.
  127. Klion AD, Law MA, Noel P, Kim YJ, Haverty TP, Nutman TB: Safety and efficacy of the monoclonal anti-interleukin-5 antibody SCH55700 in the treatment of patients with hypereosinophilic syndrome. Blood 2004;103:2939–2941.
  128. Pitini V, Teti D, Arrigo C, Righi M: Alemtuzumab therapy for refractory idiopathic hypereosinophilic syndrome with abnormal T cells: A case report. Br J Haematol 2004;127:477.
  129. Sefcick A, Sowter D, DasGupta E, Russell NH, Byrne JL: Alemtuzumab therapy for refractory idiopathic hypereosinophilic syndrome. Br J Haematol 2004;124:558–559.
  130. Archimbaud E, Guyotat D, Guillaume C, Godard J, Fiere D: Hypereosinophilic syndrome with multiple organ dysfunction treated by allogeneic bone marrow transplantation. Am J Hematol 1988;27:302–303.
  131. Sigmund DA, Flessa HC: Hypereosinophilic syndrome: Successful allogeneic bone marrow transplantation. Bone Marrow Transplant 1995;15:647–648.
  132. Fukushima T, Kuriyama K, Ito H, Miyazaki Y, Arimura K, Hata T, Saitoh M, Tomonaga M: Successful bone marrow transplantation for idiopathic hypereosinophilic syndrome. Br J Haematol 1995;90:213–215.
  133. Esteva-Lorenzo FJ, Meehan KR, Spitzer TR, Mazumder A: Allogeneic bone marrow transplantation in a patient with hypereosinophilic syndrome. Am J Hematol 1996;51:164–165.
  134. Sadoun A, Lacotte L, Delwail V, Randriamalala E, Patri S, Babin P, Brizard A, Guilhot F: Allogeneic bone marrow transplantation for hypereosinophilic syndrome with advanced myelofibrosis. Bone Marrow Transplant 1997;19:741–743.
  135. Chockalingam A, Jalil A, Shadduck RK, Lister J: Allogeneic peripheral blood stem cell transplantation for hypereosinophilic syndrome with severe cardiac dysfunction. Bone Marrow Transplant 1999;23:1093–1094.
  136. Vazquez L, Caballero D, Canizo CD, Lopez C, Hernandez R, Gonzalez I, Flores T, San Miguel JF: Allogeneic peripheral blood cell transplantation for hypereosinophilic syndrome with myelofibrosis. Bone Marrow Transplant 2000;25:217–218.
  137. Juvonen E, Volin L, Koponen A, Ruutu T: Allogeneic blood stem cell transplantation following non-myeloablative conditioning for hypereosinophilic syndrome. Bone Marrow Transplant 2002;29:457–458.
  138. Ueno NT, Anagnostopoulos A, Rondon G, Champlin RE, Mikhailova N, Pankratova OS, Zoubarovskaya LS, Semenova EV, Afanasyev BV, O’Brien S, Andreeff M, Zaritskey AY: Successful non-myeloablative allogeneic transplantation for treatment of idiopathic hypereosinophilic syndrome. Br J Haematol 2002;119:131–134.
  139. Cooper MA, Akard LP, Thompson JM, Dugan MJ, Jansen J: Hypereosinophilic syndrome: Long-term remission following allogeneic stem cell transplant in spite of transient eosinophilia post-transplant. Am J Hematol 2004;78:33–36.
  140. Ishii Y, Ito Y, Kuriyama Y, Tauchi T, Ohyashiki K: Successful treatment with imatinib mesylate of hypereosinophilic syndrome (chronic eosinophilic leukemia) with myelofibrosis. Leuk Res 2004;28(suppl 1):79–80.

    External Resources

  141. Yamaguchi Y, Hayashi Y, Sugama Y, Miura Y, Kasahara T, Kitamura S, Torisu M, Mita S, Tominaga A, Takatsu K: Highly purified murine interleukin 5 (IL-5) stimulates eosinophil function and prolongs in vitro survival. IL-5 as an eosinophil chemotactic factor. J Exp Med 1988;167:1737–1742.
  142. Elsner J, Hochstetter R, Spiekermann K, Kapp A: Surface and mRNA expression of the CD52 antigen by human eosinophils but not by neutrophils. Blood 1996;88:4684–4693.


Pay-per-View Options
Direct payment This item at the regular price: USD 38.00
Payment from account With a Karger Pay-per-View account (down payment USD 150) you profit from a special rate for this and other single items.
This item at the discounted price: USD 26.50