Hypereosinophilic Syndrome Follow-up
- Author: Venkata Anuradha Samavedi, MBBS, MD; Chief Editor: Emmanuel C Besa, MD more...
Hypereosinophilic syndrome has many and varied complications. Their development depends entirely on which organ systems are involved in the disease process (see Presentation and Pathophysiology). The most serious complication of hypereosinophilic syndrome is cardiac involvement, which can lead to myocardial fibrosis, chronic heart failure, and death (see Pathophysiology).
Hypereosinophilic syndrome carries a variable prognosis. It is a chronic and progressive disorder that is potentially fatal if left untreated. Blast transformation may occur after many years.
Although initial studies of hypereosinophilic syndrome showed a very poor prognosis (a 3-y survival rate of 12%), management of cardiovascular disease by early echocardiographic monitoring 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%.
The availability of tyrosine kinase inhibitors such as imatinib, which prevent progression of cardiac disease and other organ damage—particularly in FIP1L1/PGDFRA–positive cases—will likely further improve the prognosis of hypereosinophilic syndrome. 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 the following:
Elevated serum IgE level
Sustained response to corticosteroids
Early diagnosis and intensive management
The presence of features that are suggestive of myeloproliferative disorder (MPD) and leukocytosis greater than 90,000/μ L carry a worse prognosis in hypereosinophilic syndrome.
Periodically observe patients with hypereosinophilic syndrome to confirm that the eosinophilia is controlled and that no evidence of new or worsening organ involvement occurs. Patients should be advised to report any new or worsening symptoms.
Follow up patients with hypereosinophilic syndrome with serum troponin levels every 3 months, as well as with echocardiograms and pulmonary function tests every 6 months.
Seifert M, Gerth J, Gajda M, et al. [Eosinophilia - a challenging differential diagnosis] [German]. Med Klin (Munich). 2008 Aug 15. 103(8):591-7. [Medline].
Galli SJ, Goetzl EJ. Eosinophils, basophils, and mast cells. Handin RI, Stossel TP, Lux SE, Stossel TP, eds. Blood: Principles and Practice of Hematology. Baltimore, Md: Lippincott Williams & Wilkins; 1995. 621-40.
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. 2006 Jun. 117(6):1292-302. [Medline].
Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975 Jan. 54(1):1-27. [Medline].
Simon HU, Rothenberg ME, Bochner BS, Weller PF, Wardlaw AJ, Wechsler ME, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol. 2010 Jul. 126(1):45-9. [Medline].
Klion AD. Eosinophilic myeloproliferative disorders. Hematology Am Soc Hematol Educ Program. 2011. 2011:257-63. [Medline].
Liao W, Long H, Chang CC, Lu Q. The Eosinophil in Health and Disease: from Bench to Bedside and Back. Clin Rev Allergy Immunol. 2015 Sep 26. [Medline].
Hardy WR, Anderson RE. The hypereosinophilic syndromes. Ann Intern Med. 1968 Jun. 68(6):1220-9. [Medline].
Wardlaw AJ, Kay AB. Eosinopenia and eosinophilia. Beutler E, Lichtman MA, Coller BS, Kipps TJ, eds. Williams Hematology. 5th ed. New York, NY: McGraw- Hill; 1995. 844-52.
Tefferi A, Patnaik MM, Pardanani A. Eosinophilia: secondary, clonal and idiopathic. Br J Haematol. 2006 Jun. 133(5):468-92. [Medline].
Rothenberg ME. Eosinophilia. N Engl J Med. 1998 May 28. 338(22):1592-600. [Medline].
Gleich GJ, Leiferman KM. The hypereosinophilic syndromes: current concepts and treatments. Br J Haematol. 2009 May. 145(3):271-85. [Medline].
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. 2009 Sep. 94(9):1236-41. [Medline]. [Full Text].
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. 2008 Oct. 23(10):1713-8. [Medline].
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. 2008 Sep 15. 112(6):2500-7. [Medline].
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. 1996 Feb 15. 87(4):1416-22. [Medline]. [Full Text].
O'Connell EM, Nutman TB. Eosinophilia in Infectious Diseases. Immunol Allergy Clin North Am. 2015 Aug. 35 (3):493-522. [Medline].
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].
Klion A. Hypereosinophilic syndrome: current approach to diagnosis and treatment. Annu Rev Med. 2009. 60:293-306. [Medline].
Schreiber A, LeBlanc M, Thibeault MM, Mathieu S. Generalized Recalcitrant Pruritus as the Presenting Manifestation of Hypereosinophilic Syndrome. J Cutan Med Surg. 2016 Jan 29. [Medline].
Curtis C, Ogbogu PU. Evaluation and Differential Diagnosis of Persistent Marked Eosinophilia. Immunol Allergy Clin North Am. 2015 Aug. 35 (3):387-402. [Medline].
Adams JC, Dal-Bianco JP, Kumar G, Callahan MJ. Hypereosinophilic syndrome with characteristic left ventricular thrombus demonstrated by contrast echocardiography. Neth Heart J. 2009 Apr. 17(4):169-70. [Medline]. [Full Text].
Fauci AS, Harley JB, Roberts WC, et al. NIH conference. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med. 1982 Jul. 97(1):78-92. [Medline].
Kuchynka P, Palecek T, Masek M, Cerny V, Lambert L, Vitkova I, et al. Current Diagnostic and Therapeutic Aspects of Eosinophilic Myocarditis. Biomed Res Int. 2016. 2016:2829583. [Medline].
Roufosse F. Management of Hypereosinophilic Syndromes. Immunol Allergy Clin North Am. 2015 Aug. 35 (3):561-75. [Medline].
Parrillo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic syndrome. Ann Intern Med. 1978 Aug. 89(2):167-72. [Medline].
Khoury P, Desmond R, Pabon A, Holland-Thomas N, Ware JM, Arthur DC, et al. Clinical features predict responsiveness to imatinib in platelet derived growth factor receptor alpha-negative hypereosinophilic syndrome. Allergy. 2016 Jan 21. [Medline].
Luciano L, Catalano L, Sarrantonio C, et al. AlphaIFN-induced hematologic and cytogenetic remission in chronic eosinophilic leukemia with t(1;5). Haematologica. 1999 Jul. 84(7):651-3. [Medline]. [Full Text].
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. 1998 Jun. 58(2):137-41. [Medline]. [Full Text].
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. 1996 Jan. 92(1):176-83. [Medline].
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. 2009 Jan 1. 15(1):368-73. [Medline].
Schwartz LB, Sheikh J, Singh A. Current strategies in the management of hypereosinophilic syndrome, including mepolizumab. Curr Med Res Opin. 2010 Aug. 26(8):1933-46. [Medline].
Strati P, Cortes J, Faderl S, Kantarjian H, Verstovsek S. Long-term follow-up of patients with hypereosinophilic syndrome treated with Alemtuzumab, an anti-CD52 antibody. Clin Lymphoma Myeloma Leuk. 2013 Jun. 13(3):287-91. [Medline].
Roufosse FE, Kahn JE, Gleich GJ, Schwartz LB, Singh AD, Rosenwasser LJ, et al. Long-term safety of mepolizumab for the treatment of hypereosinophilic syndromes. J Allergy Clin Immunol. 2013 Feb. 131(2):461-7.e1-5. [Medline]. [Full Text].
Bain BJ. Eosinophilia--idiopathic or not?. N Engl J Med. 1999 Oct 7. 341(15):1141-3. [Medline].
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. 1998 Dec. 107(2):111-7. [Medline].
Weller PF. The idiopathic hypereosinophilic syndrome. Arch Dermatol. 1996 May. 132(5):583-5. [Medline].
Hsieh FH. Hypereosinophilic syndrome. Ann Allergy Asthma Immunol. 2014 Jun. 112(6):484-8. [Medline].
Schwartz RS. The hypereosinophilic syndrome and the biology of cancer. N Engl J Med. 2003 Mar 27. 348(13):1199-200. [Medline].