Clinical analysis of 15 cases of idiopathic hypereosinophilic syndrome_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

DU Gaojie ^1,2 ,  ZHANG Yongming ² , LIN Jiangtao ² , ZHAO Ling ³

1. Department of Respiratory Diseases, Changli People’s Hospital, Qinhuangdao, Hebei 066600, P. R. China;
2. Department of Pulmonary and Critical Care Medicine, China-Japan Friendship Hospital, Beijing 100029, P. R. China;
3. Department of Pathology, China-Japan Friendship Hospital, Beijing 100029, P. R. China;

Corresponding?author：

ZHANG Yongming, Email: cacpzym@163.com

Keywords：

Idiopathic hypereosinophilic syndrome; Broncho-alveolar lavage fluid; Computed tomography; Glucocorticoid treatment

DOI：

10.7507/1671-6205.201812036

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Objective To explore the clinical characteristics of idiopathic hypereosinophilic syndrome (IHES), and improve the early diagnosis and treatment of such diseases.Methods The clinical diagnosis and treatment data were retrospectively analyzed from the patients with confirmed IHES hospitalized in China-Japan Friendship Hospital between September 2010 to May 2018.Results Fifteen patients were included. There were 3 women and 12 men in the study, with an average age of 53.7±21.3 years. Eleven patients had respiratory problems, with an average course of 7 months. Most lesions occurred in both lungs. Patchy distribution, ground glass opacity, pleural effusion and mediastinal lymph node enlargement were common in the chest computed tomography. Serum total IgE was significantly increased. Four patients had other systems involved rather than respiratory system. One of them had digestive problems and another 3 had skin diseases. There was a significant increase in eosinophils in peripheral blood, bone marrow and histopathology, the same as eosinophils in sputum, pleural effusion, and bronchoalveolar lavage fluid. Hypoxemia was common in patients with respiratory problems. The blood eosinophil and total IgE were reduced after glucocorticoid treatment, and the hypoxemia was significantly improved.Conclusions The clinical symptoms, signs and image of chest computed tomography are not specific in IHES, so the rate of misdiagnosis and wrong diagnosis is high. This disease involves many organs or systems, so the pathological examination should be completed as soon as possible to make a clear diagnosis to prevent further damage. Glucocorticoid treatment is effective in this disease.

Citation： DU Gaojie, ZHANG Yongming, LIN Jiangtao, ZHAO Ling. Clinical analysis of 15 cases of idiopathic hypereosinophilic syndrome. Chinese Journal of Respiratory and Critical Care Medicine, 2020, 19(1): 59-63. doi: 10.7507/1671-6205.201812036 Copy

1.	Fauci AS, Harley JB, Roberts WC, et al. NIH conference. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med, 1982, 97(1): 78-92.
2.	曲士強. 單中心 60 例高嗜酸性粒細胞綜合征的臨床特征及長期療效. 中華血液雜志, 2016, 37(10): 881-885.
3.	Simon HU, Rothenberg ME, Bochner BS, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol, 2010, 126(1): 45-49.
4.	Valent P, Klion AD, Rosenwasser LJ, et al. ICON: Eosinophil disorders. World Allergy Organ J, 2012, 5(12): 174-181.
5.	陳嘉林. 對 EOS 增多癥的認識. 中華全科醫師雜志, 2014, 13(11): 891-892.
6.	Sivasankri P, Ramesh A, Sampath V. Hypereosinophilic syndrome masquerading as acrodermatitis enteropathica. Indian J Dermatol Venereol Leprol, 2019, 85(4): 418-422.
7.	Abayazeed RM, Abdel-Hay MA, Elfwal S, et al. A case report of L?ffler endocarditis in idiopathic hypereosinophilic syndrome: recovery is possible. Eur Heart J Case Rep, 2018, 2(2): yty030.
8.	Gotlib J. World Health Organization-defined eosinophilic disorders: 2014 update on diagnosis, risk stratification, and management. Am J Hematol, 2014, 89(3): 325-337.
9.	Gotlib J. World Health Organization‐defined eosinophilic disorders: 2017 update on diagnosis, risk stratification, and management. Hematology, 2017, 92(11): 1243-1259.
10.	Sweidan AJ, Brys A, Sohn D, et al. Diagnostic and therapeutic considerations in idiopathic hypereosinophilia with warm autoimmune hemolytic anemia. J Blood Med, 2015, 6: 257-260.
11.	Khalid F, Holguin F. Idiopathic hypereosinophilic syndrome in an elderly female: a case report. Am J Case Rep, 2019, 20: 381-384.
12.	Lim KS, Ko J, Lee SS, et al. A case of idiopathic hypereosinophilic syndrome presenting with acute respiratory distress syndrome. Allergy Asthma Immunol Res, 2014, 6(1): 98-101.
13.	Dulohery MM, Patel RR, Schneider F, et al. Lung involvement in hypereosinophilic syndromes. Respir Med, 2011, 105(1): 114-121.
14.	Ibe T, Wada H, Sakakura K. A case of pulmonary hypertension associated with idiopathic hypereosinophilic syndrome. Int Heart J, 2018, 59(4): 887-890.
15.	章星琪. 嗜酸性粒細胞增多綜合征和嗜酸性皮膚病. 國際皮膚性病學雜志, 2012, 8(12): 133-136.
16.	Kuang FL, Klion AD. Biologic agents for the treatment of hypereosinophilic syndromes. J Allergy Clin Immunol Pract, 2017, 5(6): 1502-1509.

1. Fauci AS, Harley JB, Roberts WC, et al. NIH conference. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med, 1982, 97(1): 78-92.
2. 曲士強. 單中心 60 例高嗜酸性粒細胞綜合征的臨床特征及長期療效. 中華血液雜志, 2016, 37(10): 881-885.
3. Simon HU, Rothenberg ME, Bochner BS, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol, 2010, 126(1): 45-49.
4. Valent P, Klion AD, Rosenwasser LJ, et al. ICON: Eosinophil disorders. World Allergy Organ J, 2012, 5(12): 174-181.
5. 陳嘉林. 對 EOS 增多癥的認識. 中華全科醫師雜志, 2014, 13(11): 891-892.
6. Sivasankri P, Ramesh A, Sampath V. Hypereosinophilic syndrome masquerading as acrodermatitis enteropathica. Indian J Dermatol Venereol Leprol, 2019, 85(4): 418-422.
7. Abayazeed RM, Abdel-Hay MA, Elfwal S, et al. A case report of L?ffler endocarditis in idiopathic hypereosinophilic syndrome: recovery is possible. Eur Heart J Case Rep, 2018, 2(2): yty030.
8. Gotlib J. World Health Organization-defined eosinophilic disorders: 2014 update on diagnosis, risk stratification, and management. Am J Hematol, 2014, 89(3): 325-337.
9. Gotlib J. World Health Organization‐defined eosinophilic disorders: 2017 update on diagnosis, risk stratification, and management. Hematology, 2017, 92(11): 1243-1259.
10. Sweidan AJ, Brys A, Sohn D, et al. Diagnostic and therapeutic considerations in idiopathic hypereosinophilia with warm autoimmune hemolytic anemia. J Blood Med, 2015, 6: 257-260.
11. Khalid F, Holguin F. Idiopathic hypereosinophilic syndrome in an elderly female: a case report. Am J Case Rep, 2019, 20: 381-384.
12. Lim KS, Ko J, Lee SS, et al. A case of idiopathic hypereosinophilic syndrome presenting with acute respiratory distress syndrome. Allergy Asthma Immunol Res, 2014, 6(1): 98-101.
13. Dulohery MM, Patel RR, Schneider F, et al. Lung involvement in hypereosinophilic syndromes. Respir Med, 2011, 105(1): 114-121.
14. Ibe T, Wada H, Sakakura K. A case of pulmonary hypertension associated with idiopathic hypereosinophilic syndrome. Int Heart J, 2018, 59(4): 887-890.
15. 章星琪. 嗜酸性粒細胞增多綜合征和嗜酸性皮膚病. 國際皮膚性病學雜志, 2012, 8(12): 133-136.
16. Kuang FL, Klion AD. Biologic agents for the treatment of hypereosinophilic syndromes. J Allergy Clin Immunol Pract, 2017, 5(6): 1502-1509.

Chinese Journal of Respiratory and Critical Care Medicine

Clinical analysis of 15 cases of idiopathic hypereosinophilic syndrome

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