eMedicine Specialties > Hematology > Immune System and Disorders

Hypereosinophilic Syndrome: Follow-up

Author: Venkata Samavedi, MBBS, MD, Internist in Houston, TX
Coauthor(s): Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center; Vincent E Herrin, MD, Associate Professor of Medicine, Divisions of Hematology and Oncology, University of Mississippi School of Medicine; Joe C Files, MD, Director, Division of Hematology, Associate Chairman, Professor, Department of Internal Medicine, University of Mississippi Medical Center; Youwen Zhou, MD, PhD, FRCP(C), Associate Professor, Department of Dermatology and Skin Science, University of British Columbia; Director, Hyperhidrosis Specialty Clinic, Co-Director, Psoriasis and Phototherapy Centre, Consulting Physician, Department of Dermatology, Vancouver General Hospital, Co-Director, Vitiligo and Pigmentation Clinic, Oncologist Consultant, Skin Tumor Program, BC Cancer Agency; Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Contributor Information and Disclosures

Updated: Oct 4, 2009

Follow-up

Further Outpatient Care

  • Periodically observe patients with hypereosinophilic syndrome to confirm that the eosinophilia is controlled and that no evidence of new or worsening organ involvement occurs.
  • Follow up patients with hypereosinophilic syndrome with serum troponin levels every 3 months, as well as with ECHO and pulmonary function tests every 6 months.

Deterrence/Prevention

  • No known method of prevention for hypereosinophilic syndrome exists.

Complications

  • Many varied complications of hypereosinophilic syndrome depend entirely on the organ systems that are involved in the disease process (see Clinical and Pathophysiology).
  • The most serious complication of hypereosinophilic syndrome is cardiac involvement that leads to myocardial fibrosis, CHF, and death (see Pathophysiology).

Prognosis

  • Hypereosinophilic syndrome carries a variable prognosis. It is a chronic and progressive disorder which is potentially fatal if left untreated. Blast transformation could occur after many years.
  • Although initial studies for hypereosinophilic syndrome showed a very poor prognosis (a 3-y survival rate of 12%),4 management of cardiovascular disease by early monitoring via ECHO and advances in medical and surgical therapies have improved the overall survival. A study of 40 cases by Lefebcve et al showed a 5-year survival of 80% and a 15-year survival of 42%.22 As discussed above, due to the availability of tyrosine kinase inhibitors, which prevent progression of cardiac disease and other organ damage—particularly in FIP1L1/PGDFRA– positive cases—these agents will likely further improve the prognosis of hypereosinophilic syndrome (see Treatment, Medical Care, Tyrosine kinase inhibitors). However, FIP1L1/PGDFRA– negative cases of hypereosinophilic syndrome that are resistant to corticosteroids have not been shown to have a durable response to imatinib.Lastly, additional insight into the molecular pathogenesis of such cases of hypereosinophilic syndrome is required to develop effective targeted therapies.
  • Features that indicate a favorable prognosis in hypereosinophilic syndrome include angioedema, urticaria, an elevated serum IgE level, a sustained response to corticosteroids, early diagnosis, and intensive management.
  • The presence of features that are suggestive of MPD and luekocytosis >90,000 carry a worse prognosis in hypereosinophilic syndrome.

Patient Education

  • Individuals with hypereosinophilic syndrome should report any new or worsening symptoms.
  • Hypereosinophilic syndrome may involve almost any organ system, and the prognosis is variable.

Miscellaneous

Medicolegal Pitfalls

  • Failure to make the diagnosis of hypereosinophilic syndrome is a pitfall. Because the onset of hypereosinophilic syndrome is often insidious, the diagnosis may be overlooked until significant end-organ impairment has occurred.
 


More on Hypereosinophilic Syndrome

Overview: Hypereosinophilic Syndrome
Differential Diagnoses & Workup: Hypereosinophilic Syndrome
Treatment & Medication: Hypereosinophilic Syndrome
Follow-up: Hypereosinophilic Syndrome
Multimedia: Hypereosinophilic Syndrome
References
Further Reading
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References

  1. Seifert M, Gerth J, Gajda M, et al. [Eosinophilia - a challenging differential diagnosis] [German]. Med Klin (Munich). Aug 15 2008;103(8):591-7. [Medline].

  2. Galli SJ, Goetzl EJ. Eosinophils, basophils, and mast cells. In: Handin RI, Stossel TP, Lux SE, Stossel TP, eds. Blood: Principles and Practice of Hematology. Baltimore, Md: Lippincott Williams & Wilkins; 1995:621-40.

  3. Klion AD, Bochner BS, Gleich GJ, et al, and The Hypereosinophilic Syndromes Working Group. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol. Jun 2006;117(6):1292-302. [Medline].

  4. Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). Jan 1975;54(1):1-27. [Medline].

  5. Hardy WR, Anderson RE. The hypereosinophilic syndromes. Ann Intern Med. Jun 1968;68(6):1220-9. [Medline].

  6. Wardlaw AJ, Kay AB. Eosinopenia and eosinophilia. In: Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw- Hill; 1995:844-52.

  7. Tefferi A, Patnaik MM, Pardanani A. Eosinophilia: secondary, clonal and idiopathic. Br J Haematol. Jun 2006;133(5):468-92. [Medline].

  8. Rothenberg ME. Eosinophilia. N Engl J Med. May 28 1998;338(22):1592-600. [Medline].

  9. Gleich GJ, Leiferman KM. The hypereosinophilic syndromes: current concepts and treatments. Br J Haematol. May 2009;145(3):271-85. [Medline].

  10. Helbig G, Wieczorkiewicz A, Dziaczkowska-Suszek J, Majewski M, Kyrcz-Krzemien S. T-cell abnormalities are present at high frequencies in patients with hypereosinophilic syndrome. Haematologica. Sep 2009;94(9):1236-41. [Medline].

  11. Cincin AA, Ozben B, Tanrikulu MA, Baskan O, Agirbasli M. Large apical thrombus in a patient with persistent heart failure and hypereosinophilia: Löffler endocarditis. J Gen Intern Med. Oct 2008;23(10):1713-8. [Medline].

  12. Bain B. The idiopathic hypereosinophilic syndrome and eosinophilic leukemias. Haematologica. Feb 2004;89(2):133-7. [Medline]. [Full Text].

  13. Bain BJ. Eosinophilia--idiopathic or not?. N Engl J Med. Oct 7 1999;341(15):1141-3. [Medline].

  14. Oliver JW, Deol I, Morgan DL, Tonk VS. Chronic eosinophilic leukemia and hypereosinophilic syndromes. Proposal for classification, literature review, and report of a case with a unique chromosomal abnormality. Cancer Genet Cytogenet. Dec 1998;107(2):111-7. [Medline].

  15. Weller PF. The idiopathic hypereosinophilic syndrome. Arch Dermatol. May 1996;132(5):583-5. [Medline].

  16. Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. May 15 1994;83(10):2759-79. [Medline]. [Full Text].

  17. Fauci AS, Harley JB, Roberts WC, et al. NIH conference. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med. Jul 1982;97(1):78-92. [Medline].

  18. Yamada Y, Sanchez-Aguilera A, Brandt EB, et al. FIP1L1/PDGFRalpha synergizes with SCF to induce systemic mastocytosis in a murine model of chronic eosinophilic leukemia/hypereosinophilic syndrome. Blood. Sep 15 2008;112(6):2500-7. [Medline].

  19. Simon HU, Plötz SG, Dummer R, Blaser K. Abnormal clones of T cells producing interleukin-5 in idiopathic eosinophilia. N Engl J Med. Oct 7 1999;341(15):1112-20. [Medline]. [Full Text].

  20. Brugnoni D, Airó P, Rossi G, et al. A case of hypereosinophilic syndrome is associated with the expansion of a CD3-CD4+ T-cell population able to secrete large amounts of interleukin-5. Blood. Feb 15 1996;87(4):1416-22. [Medline]. [Full Text].

  21. Bain BJ. Relationship between idiopathic hypereosinophilic syndrome, eosinophilic leukemia, and systemic mastocytosis. Am J Hematol. Sep 2004;77(1):82-5. [Medline]. [Full Text].

  22. Lefebvre C, Bletry O, Degoulet P, et al. [Prognostic factors of hypereosinophilic syndrome. Study of 40 cases] [French]. Ann Med Interne (Paris). 1989;140(4):253-7. [Medline].

  23. Klion A. Hypereosinophilic syndrome: current approach to diagnosis and treatment. Annu Rev Med. 2009;60:293-306. [Medline].

  24. Adams JC, Dal-Bianco JP, Kumar G, Callahan MJ. Hypereosinophilic syndrome with characteristic left ventricular thrombus demonstrated by contrast echocardiography. Neth Heart J. Apr 2009;17(4):169-70. [Medline].

  25. Parrillo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic syndrome. Ann Intern Med. Aug 1978;89(2):167-72. [Medline].

  26. Luciano L, Catalano L, Sarrantonio C, et al. AlphaIFN-induced hematologic and cytogenetic remission in chronic eosinophilic leukemia with t(1;5). Haematologica. Jul 1999;84(7):651-3. [Medline]. [Full Text].

  27. Yamada O, Kitahara K, Imamura K, et al. 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. Jun 1998;58(2):137-41. [Medline]. [Full Text].

  28. Malbrain ML, Van den Bergh H, Zachée 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. Jan 1996;92(1):176-83. [Medline].

  29. Verstovsek S, Tefferi A, Kantarjian H, Manshouri T, Luthra R, Pardanani A, et al. Alemtuzumab therapy for hypereosinophilic syndrome and chronic eosinophilic leukemia. Clin Cancer Res. Jan 1 2009;15(1):368-73. [Medline].

  30. Bain BJ. Hypereosinophilia. Curr Opin Hematol. Jan 2000;7(1):21-5. [Medline].

  31. Broustet A, Bernard P, Dachary D, et al. Acute eosinophilic leukemia with a translocation (10p+;11q-). Cancer Genet Cytogenet. Apr 15 1986;21(4):327-33. [Medline].

  32. Felice PV, Sawicki J, Anto J. Endomyocardial disease and eosinophilia. Angiology. Nov 1993;44(11):869-74. [Medline].

  33. Fischkoff SA, Testa JR, Schiffer CA. Acute eosinophilic leukemia with a (10;11) chromosomal translocation. Leukemia. Jun 1988;2(6):394-7. [Medline].

  34. Maubach PA, Bauchinger M, Emmerich B, Rastetter J. Trisomy 7 and 8 in Ph-negative chronic eosinophilic leukemia. Cancer Genet Cytogenet. Jun 1985;17(2):159-64. [Medline].

  35. Salmon-Nguyen F, Busson M, Daniel M, et al. CALM-AF10 fusion gene in leukemias: simple and inversion-associated translocation (10;11). Cancer Genet Cytogenet. Oct 15 2000;122(2):137-40. [Medline].

  36. Schwartz RS. The hypereosinophilic syndrome and the biology of cancer. N Engl J Med. Mar 27 2003;348(13):1199-200. [Medline].

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Keywords

hypereosinophilic syndrome, HES, idiopathic hypereosinophilic syndrome, Loeffler endocarditis, Loeffler's endocarditis, chronic eosinophilic leukemia, CEL, eosinophilia, systemic mastocytosis –associated eosinophilia, SM-CEL, FIP1L1-PDGFRA mutation, C-KIT mutation, tyrosine kinase inhibitors

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Contributor Information and Disclosures

Author

Venkata Samavedi, MBBS, MD, Internist in Houston, TX
Disclosure: Nothing to disclose.

Coauthor(s)

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

Vincent E Herrin, MD, Associate Professor of Medicine, Divisions of Hematology and Oncology, University of Mississippi School of Medicine
Vincent E Herrin, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Society of Hematology
Disclosure: Nothing to disclose.

Joe C Files, MD, Director, Division of Hematology, Associate Chairman, Professor, Department of Internal Medicine, University of Mississippi Medical Center
Joe C Files, MD is a member of the following medical societies: American Association for Cancer Education, American Association for the Advancement of Science, American College of Physicians, American Federation for Medical Research, American Heart Association, American Medical Association, American Society of Human Genetics, Mississippi State Medical Association, New York Academy of Sciences, and Southern Medical Association
Disclosure: Nothing to disclose.

Youwen Zhou, MD, PhD, FRCP(C), Associate Professor, Department of Dermatology and Skin Science, University of British Columbia; Director, Hyperhidrosis Specialty Clinic, Co-Director, Psoriasis and Phototherapy Centre, Consulting Physician, Department of Dermatology, Vancouver General Hospital, Co-Director, Vitiligo and Pigmentation Clinic, Oncologist Consultant, Skin Tumor Program, BC Cancer Agency
Youwen Zhou, MD, PhD, FRCP(C) is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Medical Editor

Antoni Ribas, MD, Department of Medicine, Division of Hematology-Oncology, Assistant Professor of Medicine, University of California at Los Angeles Medical Center
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Troy H Guthrie, Jr, MD, Director of Cancer Institute, Baptist Medical Center
Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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