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과호산구증가증 분류와 치료의 최신지견

Recent advances in the classification and management of hypereosinophilia

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주제분류소아과학

초록

Numerous disorders and etiologies may underlie increased eosinophil counts. Hypereosinophilia (HE) is defined as a peripheral blood eosinophil count greater than 1,500/mm3 and may be potentially harmful because of tissue damage. Hypereosinophilic syndrome (HES) also represents a heterogeneous disorder characterized by persistent HE with the evidence of organ dysfunction, clinical symptoms, or both caused by eosinophilia. The refining criteria and subclassification of HE and HES are currently being revised on cellular and molecular based diagnostic methods. Initial approaches focus on evaluating various underlying causes, including helminthic infections, adverse drug reactions, allergic diseases, and neoplastic diseases. When secondary causes of HE are excluded, the workup should proceed to the evaluation of primary/clonal bone marrow disease, including fip 1-like 1-platelet driven growth factor receptor alpha (FIP1L1-PDGFRA) mutation. Concurrently, if the patient has symptoms and signs, organ damage or dysfunction must be evaluated. Although, corticosteroids are the mainstay of therapy in confirmed HES, imatinib is considered a definitive treatment for FIP1L1-PDGFRA, platelet driven growth factor receptor beta rearranged HE and HES. In this article, we discuss recent advances in the classification of and practical approaches to HE and HES. In addition, we introduce several promising therapies for HE and HES.

Abstract

Numerous disorders and etiologies may underlie increased eosinophil counts. Hypereosinophilia (HE) is defined as a peripheral blood eosinophil count greater than 1,500/mm3 and may be potentially harmful because of tissue damage. Hypereosinophilic syndrome (HES) also represents a heterogeneous disorder characterized by persistent HE with the evidence of organ dysfunction, clinical symptoms, or both caused by eosinophilia. The refining criteria and subclassification of HE and HES are currently being revised on cellular and molecular based diagnostic methods. Initial approaches focus on evaluating various underlying causes, including helminthic infections, adverse drug reactions, allergic diseases, and neoplastic diseases. When secondary causes of HE are excluded, the workup should proceed to the evaluation of primary/clonal bone marrow disease, including fip 1-like 1-platelet driven growth factor receptor alpha (FIP1L1-PDGFRA) mutation. Concurrently, if the patient has symptoms and signs, organ damage or dysfunction must be evaluated. Although, corticosteroids are the mainstay of therapy in confirmed HES, imatinib is considered a definitive treatment for FIP1L1-PDGFRA, platelet driven growth factor receptor beta rearranged HE and HES. In this article, we discuss recent advances in the classification of and practical approaches to HE and HES. In addition, we introduce several promising therapies for HE and HES.

참고문헌 (42)

  1. Kita H, "Eosinophils : multifaceted biological properties and roles in health and disease", Immunol Rev, 242, pp.161~177 (2011)
  2. Hogan SP, "Eosinophils : biological properties and role in health and disease", Clin Exp Allergy, 38, pp.709~750 (2008)
  3. Valent P, "Pathogenesis and classification of eosinophil disorders : a review of recent developments in the field", Expert Rev Hematol, 5, pp.157~176 (2012)
  4. Brigden M, "Eosinophilia detected by automated blood cell counting in ambulatory North American outpatients. Incidence and clinical significance", Arch Pathol Lab Med, 121, pp.963~967 (1997)
  5. Rothenberg ME, "Eosinophilia", N Engl J Med, 338, pp.1592~1600 (1998)
  6. Chusid MJ, "The hypereosinophilic syndrome : analysis of fourteen cases with review of the literature", Medicine(Baltimore), 54, pp.1~27 (1975)
  7. Valent P, "Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes", J Allergy Clin Immunol, 130, pp.607~612.e9 (2012)
  8. 박찬선, "호산구증가증의 진단과 치료 - 한국인 환자를 위한 지침 초안", 천식 및 알레르기, 26(3), pp.186~197 (2006), 대한천식알레르기학회
  9. 최동철, "호산구증가증", 천식 및 알레르기, 31(4), pp.237~245 (2011), 대한천식알레르기학회
  10. 이진영, "The Prevalence of Toxocariasis and Diagnostic Value of Serologic Tests in Asymptomatic Korean Adults", Allergy, Asthma & Immunology Research, 7(5), pp.467~475 (2015), 대한천식알레르기학회
  11. Kwon NH, "The prevalence and diagnostic value of toxocariasis in unknown eosinophilia", Ann Hematol, 85, pp.233~238 (2006)
  12. 권남희, "호산구 증가증으로 내원한 환자에서 개회충증이 가지는 임상적 의미", 천식 및 알레르기, 25(4), pp.299~304 (2005), 대한천식알레르기학회
  13. Curtis C, "Evaluation and differential diagnosis of persistent marked eosinophilia", Immunol Allergy Clin North Am, 35, pp.387~402 (2015)
  14. Tefferi A, "Eosinophilia: secondary, clonal and idiopathic", Br J Haematol, 133, pp.468~492 (2006)
  15. Todenhofer T, "Severe paraneoplastic hypereosinophilia in metastatic renal cell carcinoma", BMC Urol, 12, pp.7~ (2012)
  16. Cools J, "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, 348, pp.1201~1214 (2003)
  17. Gotlib J., "World Health Organization-defined eosinophilic disorders: 2014 update on diagnosis, risk stratification, and management", Am J Hematol, 89, pp.325~337 (2014)
  18. Roufosse F, "Practical approach to the patient with hypereosinophilia", J Allergy Clin Immunol, 126, pp.39~44 (2010)
  19. Ogbogu PU, "Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy", J Allergy Clin Immunol, 124, pp.1319~1325.e3 (2009)
  20. Flaum MA, "A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. I. Hematologic manifestations", Blood, 58, pp.1012~1020 (1981)
  21. Akuthota P, "Spectrum of eosinophilic end-organ manifestations", Immunol Allergy Clin North Am, 35, pp.403~411 (2015)
  22. Wilkins HJ, "Hypereosinophilic syndrome : an update", Am J Hematol, 80, pp.148~157 (2005)
  23. Hsieh FH, "Hypereosinophilic syndrome", Ann Allergy Asthma Immunol, 112, pp.484~488 (2014)
  24. Roufosse F, "Clonal Th2 lymphocytes in patients with the idiopathic hypereosinophilic syndrome", Br J Haematol, 109, pp.540~548 (2000)
  25. Lefebvre C, "Prognostic factors of hypereosinophilic syndrome. Study of 40 cases", Ann Med Interne, 140, pp.253~257 (1989)
  26. Podjasek JC, "Mortality in hypereosinophilic syndrome : 19 years of experience at Mayo Clinic with a review of the literature", Leuk Res, 37, pp.392~395 (2013)
  27. Fauci AS, "NIH conference. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic, and therapeutic considerations", Ann Intern Med, 97, pp.78~92 (1982)
  28. 이상표, "개회충증의 핵심과 진단의 딜레마", 대한내과학회지, 84(2), pp.200~202 (2013), 대한내과학회[DOI]
  29. Klion AD, "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, 101, pp.4660~4666 (2003)
  30. Noguchi H, "Tissue eosinophilia and eosinophil degranulation in syndromes associated with fibrosis", Am J Pathol, 140, pp.521~528 (1992)
  31. Kato M, "Eosinophil infiltration and degranulation in normal human tissue", Anat Rec, 252, pp.418~425 (1998)
  32. Tefferi A, "Hypereosinophilic syndrome and clonal eosinophilia : point-of-care diagnostic algorithm and treatment update", Mayo Clin Proc, 85, pp.158~164 (2010)
  33. Malbrain ML, "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, 92, pp.176~183 (1996)
  34. Helbig G, "Imatinib mesylate may induce long-term clinical response in FIP1L1-PDGFRα-negative hypereosinophilic syndrome", Med Oncol, 29, pp.1073~1076 (2012)
  35. Pitini V, "Serum concentration of cardiac Troponin T in patients with hypereosinophilic syndrome treated with imatinib is predictive of adverse outcomes", Blood, 102, pp.3456~3457 (2003)
  36. Plotz SG, "Use of an anti-interleukin-5 antibody in the hypereosinophilic syndrome with eosinophilic dermatitis", N Engl J Med, 349, pp.2334~2339 (2003)
  37. Rothenberg ME, "Treatment of patients with the hypereosinophilic syndrome with mepolizumab", N Engl J Med, 358, pp.1215~1228 (2008)
  38. Roufosse FE, "Long-term safety of mepolizumab for the treatment of hypereosinophilic syndromes", J Allergy Clin Immunol, 131, pp.461~467.e5 (2013)
  39. Kolbeck R, "MEDI-563, a humanized anti-IL-5 receptor alpha mAb with enhanced antibody-dependent cell-mediated cytotoxicity function", J Allergy Clin Immunol, 125, pp.1344~1353.e2 (2010)
  40. Wilson TM, "IL-5 receptor α levels in patients with marked eosinophilia or mastocytosis", J Allergy Clin Immunol, 128, pp.1086~1092.e1-3 (2011)
  41. Legrand F, "Biologic therapies targeting eosinophils : current status and future prospects", J Allergy Clin Immunol Pract, 3, pp.167~174 (2015)
  42. Strati P, "Long-term follow-up of patients with hypereosinophilic syndrome treated with Alemtuzumab, an anti-CD52 antibody", Clin Lymphoma Myeloma Leuk, 13, pp.287~291 (2013)

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