Hypereosinophilic syndrome: Case series and review of the literature

Year 2024, Volume: 4 Issue: 1, 1 - 6, 29.01.2024

Nazif Yalçın , Aysegul Ertınmaz Ozkan , Nizameddin Koca

https://doi.org/10.56016/dahudermj.1391630

Abstract

Abstract
Hypereosinophilic Syndrome (HES) is caused by the uncontrolled proliferation of eosinophils generally associated with conditions such as allergic reactions or parasitic infections. This syndrome is characterized by excessive eosinophil production (>1500/mm3) that persists for more than six months and cannot be explained by secondary causes. HES symptoms can affect different body organs, and usually, nonspecific symptoms include fever, malaise, fatigue, rash, shortness of breath, and myalgia.
HES is a rare disease with multiorgan involvement, including the skin, joints, kidneys, vascular system, gastrointestinal tract, cardiac and pulmonary systems. The main feature of this disease is that overproduced eosinophils accumulate in organs and cause organ damage. Cardiac involvement plays a critical role in determining morbidity and mortality, and cardiac and large vessel thrombosis with severe clinical manifestations can also be observed.
Treatment aims to reduce the absolute eosinophil count, improve symptoms, and prevent disease progression. Pharmacologic therapy aims to maintain targeted eosinophil levels below 1.5 x 10^9/L (1500 cells/mcL) to reduce the symptoms of eosinophilic disease and prevent organ damage. Furthermore, indications for emergency treatment should be rapidly assessed and initiated promptly in appropriate patients.
This paper will discuss the diagnosis, clinical manifestations, treatment modalities, and management challenges of HES in detail through two rare case examples.

Keywords

hypereosinophilia, eosinophil, hypereosinophilic syndrome

Ethical Statement

ethical rules have been followed

Supporting Institution

there isn't supporting institution

Project Number

1

References

• Klion A. Hypereosinophilic syndrome: current approach to diagnosis and treatment. Annu Rev Med 2009;60:293-306.
• Suı T, Li Q, Geng L, Xu X, Li Y. A Case of Hypereosinophilic Syndrome Presenting with Multiorgan Thromboses Associated with Intestinal Obstruction. Turk J Haematol. 2013 Sep; 30(3): 311–314.
• 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; 126:45-9.
• Demirci NY, Kaplan M, Taçoy G, Türktaş H. Hypereosinophilic Syndrome Diagnosed with Acute Coronary Syndrome. Respir Case Rep 2017;6(3): 157-160.
• Kikuchi K, Minami K, Miyakawa H, Ishibashi M. Portal vein thrombosis in hypereosinophilic syndrome. Am J Gastroenterol. 2002;97:1274–1275.
• Rothenberg ME. Eosinophilia. New Engl J Med 1998;338:1592–1600.
• Pardanani A, Patnaik MM, Tefferi A. Eosinophilia: secondary, clonal and idiopathic. Br J Haematol 2006;133:468–492.
• Tefferi A, Gotlib J, Pardanani A.Hypereosinophilic Syndrome and Clonal Eosinophilia: Point-of-Care Diagnostic Algorithm and Treatment Update. Mayo Clin Proc 2010; 85: 158 - 164.
• Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. 1994;83:2759–2779.
• Keren M, Aksu K, Çiftci E, Kurt E. Lung, skin and heart involvement in a case with hypereosinophilic syndrome. Asthma Allergy Immunol 2011;9:44-5.
• Crane MM, Chang CM, Kobayashi MG, Weller PF. Incidence of myeloproliferative hypereosinophilic syndrome in the United States and an estimate of all hypereosinophilic syndrome incidence. J Allergy Clin Immunol 2010; 126:179.
• Tefferi A, Vardiman JW. Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia. 2008;22:14-22.
• Turgut B. (2012), ‘’ Hipereozinofilik Sendromlar’’ Türk Hematoloji Derneği Hematolog, 2012: 2 = 1.
• Mankad R, Bonnichsen C, Mankad S. Hypereosinophilic syndrome: cardiac diagnosis and management. Heart 2016; 102:100–6.
• https://www.uptodate.com/contents/hypereosinophilic-syndromes-clinical-manifestations pathophysiology-and diagnosis?search=hypereosinophilic%20syndrome%20diagnosis%20algoritm&source=searc result&selectedTitle=1~150&usage_type=default&display_rank=1#H6 24.07.2023 tarihli erişim
• Ogbogu PU, Bochner BS, Butterfield JH, et al. Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy. J Allergy Clin Immunol 2009; 124:1319.
• Simon HU, Plotz SG, Dummer R, Blaser K. Abnormal clones of T cells producing interleukin-5 in idiopathic eosinophilia. N Engl J Med. 1999;341:1112–1120.
• Fauci AS, Harley JB, Roberts WC, Ferrans VJ, Gralnick HR, Bjornson BH. NIH conference. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med. 1982;97:78–92.
• Wright BL, Leiferman KM, Gleich GJ. Eosinophil granule protein localization in eosinophilic endomyocardial disease. N Engl J Med 2011; 365:187.
• Wang JG, Mahmud SA, Thompson JA, et al. The principal eosinophil peroxidase product, HOSCN, is a uniquely potent phagocyte oxidant inducer of endothelial cell tissue factor activity: a potential mechanism for thrombosis in eosinophilic inflammatory states. Blood 2006; 107:558.
• Aida L, Parkhutik V, Tembl JI, et al. Embolism and impaired washout: a possible explanation of border zone strokes in hypereosinophilic syndrome. J Neurol Sci 2013; 325:162.
• Leiferman KM, Gleich GJ, Peters MS. Dermatologic manifestations of the hypereosinophilic syndromes. Immunol Allergy Clin North Am 2007; 27:415.
• 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.
• Dulohery MM, Patel RR, Schneider F, Ryu JH. Lung involvement in hypereosinophilic syndromes. Respir Med 2011; 105:114.
• Kuang FL, Klion AD. Biologic Agents for the Treatment of Hypereosinophilic Syndromes. J Allergy Clin Immunol Pract 2017; 5:1502.
• Klion AD. How I treat hypereosinophilic syndromes. Blood 2015; 126:1069.
• McMillan HJ, Johnston DL, Doja A. Watershed infarction due to acute hypereosinophilia. Neurology 2008; 70:80.
• Parrillo JE, Lawley TJ, Frank MM, et al. Immunologic reactivity in the hypereosinophilic syndrome. J Allergy Clin Immunol 1979; 64:113.
• Kobayashi M, Komatsu N, Kuwayama Y, Bandobashi K, Kubota T, Uemura Y, Taguchi H. Idiopathic hypereosinophilic syndrome presenting acute abdomen. Intern Med. 2007;46:675–678.