hypereosinophilic syndrome

Summary

Summary: A heterogeneous group of disorders with the common feature of prolonged eosinophilia of unknown cause and associated organ system dysfunction, including the heart, central nervous system, kidneys, lungs, gastrointestinal tract, and skin. There is a massive increase in the number of EOSINOPHILS in the blood, mimicking leukemia, and extensive eosinophilic infiltration of the various organs.

Top Publications

  1. Roufosse F, Kahn J, Gleich G, Schwartz L, Singh A, Rosenwasser L, et al. Long-term safety of mepolizumab for the treatment of hypereosinophilic syndromes. J Allergy Clin Immunol. 2013;131:461-7.e1-5 pubmed publisher
    ..Twenty-four subjects withdrew before study completion for death (n = 4), lack of efficacy (n = 6), or other reasons. Mepolizumab was well tolerated and effective as a long-term corticosteroid-sparing agent in PDGFRA-negative HES. ..
  2. Valent P, Klion A, Horny H, Roufosse F, Gotlib J, Weller P, et al. Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes. J Allergy Clin Immunol. 2012;130:607-612.e9 pubmed publisher
    ..secondary variants based on specific hematologic and immunologic conditions, and various forms of the hypereosinophilic syndrome. For patients in whom no underlying disease or hypereosinophilic syndrome is found, the term ..
  3. Yamada Y, Rothenberg M, Lee A, Akei H, Brandt E, Williams D, et al. The FIP1L1-PDGFRA fusion gene cooperates with IL-5 to induce murine hypereosinophilic syndrome (HES)/chronic eosinophilic leukemia (CEL)-like disease. Blood. 2006;107:4071-9 pubmed
    ..growth factor receptor alpha (PDGFRA) (F/P) fusion gene has been identified as a cause of clonal hypereosinophilic syndrome (HES), called F/P-positive chronic eosinophilic leukemia (CEL) in humans...
  4. Ackerman S, Bochner B. Mechanisms of eosinophilia in the pathogenesis of hypereosinophilic disorders. Immunol Allergy Clin North Am. 2007;27:357-75 pubmed
  5. Jin Y, Chen Q, Lu Z, Chen B, Pan J. Triptolide abrogates oncogene FIP1L1-PDGFRalpha addiction and induces apoptosis in hypereosinophilic syndrome. Cancer Sci. 2009;100:2210-7 pubmed publisher
    The pathogenesis of hypereosinophilic syndrome (HES) in some patients is highly dependent on FIP1-Like-1 (FIP1L1)-platelet-derived growth factor receptor alpha (PDGFRalpha), which can generate sustained activation signaling to maintain a ..
  6. Hosoki K, Nagao M, Iguchi K, Ihara T, Yamada Y, Higashigawa M, et al. An 8-year-old boy with hypereosinophilic syndrome. Int Arch Allergy Immunol. 2011;155 Suppl 1:117-22 pubmed publisher
    b>Hypereosinophilic syndrome (HES) is a heterogeneous group of uncommon disorders characterized by the presence of marked peripheral blood eosinophilia and tissue eosinophilia, resulting in a wide variety of clinical manifestations...
  7. Antoniu S. Novel therapies for hypereosinophilic syndromes. Neth J Med. 2010;68:304-10 pubmed
    ..Some of the novel therapies might become appropriate therapeutic options for HES. ..
  8. Rojas G, Castro D, Vigo Guevara G, Ferrua M, Barriga Maldonado V, Rotta Escalante R. [Hypereosinophilic encephalopathy with multiple cerebral infarctions in neighbouring vascular territories associated with prostate cancer]. Rev Neurol. 2006;43:762-4 pubmed
  9. Sakuta R, Tomita Y, Ohashi M, Nagai T, Murakami N. Idiopathic hypereosinophilic syndrome complicated by central sinovenous thrombosis. Brain Dev. 2007;29:182-4 pubmed
    Idiopathic hypereosinophilic syndrome (IHES) is a rare disorder affecting cardiac, pulmonary and nervous systems with peripheral neuropathy, encephalopathy and cerebral thromboembolism...

More Information

Publications74

  1. ten Oever J, Theunissen L, Tick L, Verbunt R. Cardiac involvement in hypereosinophilic syndrome. Neth J Med. 2011;69:240-4 pubmed
    b>Hypereosinophilic syndrome is a heterogeneous group of disorders characterised by hypereosinophilia and organ involvement of varying intensity...
  2. Pan J, Quintas Cardama A, Manshouri T, Giles F, Lamb P, Tefferi A, et al. The novel tyrosine kinase inhibitor EXEL-0862 induces apoptosis in human FIP1L1-PDGFR-alpha-expressing cells through caspase-3-mediated cleavage of Mcl-1. Leukemia. 2007;21:1395-404 pubmed
    ..FIP1L1)-platelet-derived growth factor receptor-alpha (FIP1L1-PDGFR-alpha) fusion kinase causes hypereosinophilic syndrome (HES) in a defined subset of patients...
  3. Grigoryan M, Geisler S, St Louis E, Baumbach G, Davis P. Cerebral arteriolar thromboembolism in idiopathic hypereosinophilic syndrome. Arch Neurol. 2009;66:528-31 pubmed publisher
    ..findings as well as postmortem brain and cardiac pathology in a patient with fulminant idiopathic hypereosinophilic syndrome. Case report. University hospital...
  4. Numagami Y, Tomita T, Murakami K, Masaki I, Kubo K, Michiharu N. Sinus thrombosis in idiopathic hypereosinophilic syndrome causing fatal cerebral haemorrhage. J Clin Neurosci. 2008;15:585-7 pubmed publisher
    Idiopathic hypereosinophilic syndrome (HES) is a leukoproliferative disorder that is characterized by sustained overproduction of eosinophils and a trend towards damage to specific organs, usually the cardiovascular system...
  5. Fletcher S, Bain B. Diagnosis and treatment of hypereosinophilic syndromes. Curr Opin Hematol. 2007;14:37-42 pubmed
    ..We also aim to discuss in more detail the diagnosis and management of various hypereosinophilic syndromes including the clonal eosinophilias and those driven by abnormal cytokine-secreting T cells...
  6. Hacker H, Chi L, Rehg J, Redecke V. NIK prevents the development of hypereosinophilic syndrome-like disease in mice independent of IKK? activation. J Immunol. 2012;188:4602-10 pubmed publisher
    ..b>Hypereosinophilic syndrome (HES) represents a group of inflammatory diseases that is characterized by increased numbers of ..
  7. Schwartz L, Sheikh J, Singh A. Current strategies in the management of hypereosinophilic syndrome, including mepolizumab. Curr Med Res Opin. 2010;26:1933-46 pubmed publisher
    Patients with hypereosinophilic syndrome (HES) vary considerably in their clinical presentation with regard to the severity and pattern of end-organ involvement...
  8. Frigui M, Hmida M, Jallouli M, Kechaou M, Frikha F, Bahloul Z. Membranous glomerulopathy associated with idiopathic hypereosinophilic syndrome. Saudi J Kidney Dis Transpl. 2010;21:320-2 pubmed
    ..We reported a case of a 52-year-old man with idiopathic hypereosinophilic syndrome associated with membranous glomerulopathy...
  9. Galimberti S, Ciabatti E, Ottimo F, Rossi A, Trombi L, Carulli G, et al. Cell clonality in hypereosinophilic syndrome: what pathogenetic role?. Clin Exp Rheumatol. 2007;25:17-22 pubmed
    Idiopathic hypereosinophilic syndrome (HES) is a heterogeneous disorder, including either a myeloproliferative or a lymphoproliferative variant (l-HES)...
  10. Klion A. How I treat hypereosinophilic syndromes. Blood. 2009;114:3736-41 pubmed publisher
    ..5 x 10(9)/L (1500/mm3) or higher and discusses the role of currently available therapeutic agents in the treatment of these patients...
  11. Choi W, Park Y, Paik K, Chang Y, Lee S, Ryoo B, et al. Peripheral T-cell lymphoma-unspecified (PTCL-U) presenting with hypereosinophilic syndrome and pleural effusions. Korean J Intern Med. 2006;21:57-61 pubmed
    b>Hypereosinophilic syndrome (HES) is a clinical disorder characterized by persistent eosinophilia and systemic involvement, in which a specific causative factor for the eosinophilia cannot be verified during a certain period of time...
  12. Kahn J, Dutoit Lefevre V, Duban Deweer S, Chafey P, Pottiez G, Lefranc D, et al. Comparative proteomic analysis of blood eosinophils reveals redox signaling modifications in patients with FIP1L1-PDGFRA-associated chronic eosinophilic leukemia. J Proteome Res. 2011;10:1468-80 pubmed publisher
    The FIP1L1-PDGFRA (F/P) fusion gene, which was identified as a recurrent molecular finding in hypereosinophilic syndrome (HES), lead to a constitutively increased tyrosine kinase activity of the fusion protein...
  13. 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;94:1236-41 pubmed publisher
    ..of eosinophilopoietic cytokines, identified by clonal rearrangement of the T-cell receptor and by the presence of aberrant T-cell immunophenotype in peripheral blood defines lymphocytic variant of hypereosinophilic syndrome (L-HES).
  14. Lierman E, Folens C, Stover E, Mentens N, Van Miegroet H, Scheers W, et al. Sorafenib is a potent inhibitor of FIP1L1-PDGFRalpha and the imatinib-resistant FIP1L1-PDGFRalpha T674I mutant. Blood. 2006;108:1374-6 pubmed
    ..Our data suggest that low doses of sorafenib could be efficient for the treatment of FIP1L1-PDGFRA-positive CEL and could be used to overcome resistance to imatinib associated with the T674I mutation...
  15. Tefferi A, Gotlib J, Pardanani A. Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update. Mayo Clin Proc. 2010;85:158-64 pubmed publisher
    ..b>Hypereosinophilic syndrome, a subcategory of idiopathic eosinophilia, is defined by the presence of a peripheral blood eosinophil ..
  16. Roufosse F, de Lavareille A, Schandene L, Cogan E, Georgelas A, WAGNER L, et al. Mepolizumab as a corticosteroid-sparing agent in lymphocytic variant hypereosinophilic syndrome. J Allergy Clin Immunol. 2010;126:828-835.e3 pubmed publisher
    ..agent for patients with Fip1-like 1/platelet-derived growth factor receptor ? fusion (F/P)-negative hypereosinophilic syndrome (HES)...
  17. Kobayashi M, Kubota T, Uemura Y, Taguchi H. A case of hypereosinophilic syndrome presenting with chronic cough successfully treated with imatinib. Respirology. 2009;14:302-4 pubmed publisher
    ..We describe the case of a 42-year-old man with hypereosinophilic syndrome presenting with chronic dry cough...
  18. Bozcali E, Aliyev F, Agac M, Erkan H, Okcun B, Babalik E, et al. Unusual case of aortic valve involvement in patient with Löffler's endomyocarditis: management, follow-up and short review of the literature. J Thromb Thrombolysis. 2007;24:309-13 pubmed
    Idiopathic hypereosinophilic syndrome (IHES) is an uncommon systemic disease which is characterised by blood eosinophilia and multiple clinical presentations. Cardiac involvement is the major cause of mortality and morbidity...
  19. Srinivasan A, Lavanya R, Sankar J. Steroid-unresponsive hypereosinophilic syndrome. Ann Trop Paediatr. 2011;31:273-7 pubmed publisher
    b>Hypereosinophilic syndrome in children is rare and is characterised by sustained over-production of eosinophils. An 11-year-old boy was diagnosed with hypereosinophilic syndrome as per standard criteria...
  20. Ayhan A, Timur C, Ayhan Y, Cakır B, Erguven M. Does hypereosinophilic syndrome precede common B acute lymphoblastic leukaemia in childhood? A case report. Acta Haematol. 2012;127:90-2 pubmed publisher
    b>Hypereosinophilic syndrome (HES) and the association of hypereosinophilia with acute lymphoblastic leukaemia (ALL) are both rare in children...
  21. Cheung Y, Wong S, Lam P, Lee K, Wong J, Lai P. Spontaneous liver rupture in hypereosinophilic syndrome: a rare but fatal complication. World J Gastroenterol. 2009;15:5875-8 pubmed
    We report a rare case of spontaneous liver rupture in a patient with hypereosinophilic syndrome (HES), of which the diagnosis was delayed, resulting in a fatal outcome...
  22. Roufosse F, Goldman M, Cogan E. Hypereosinophilic syndromes. Orphanet J Rare Dis. 2007;2:37 pubmed
    ..Prognosis has improved significantly since definition of HES, and currently depends on development of irreversible heart failure, as well as eventual malignant transformation of myeloid or lymphoid cells...
  23. Salemi S, Yousefi S, Simon D, Schmid I, Moretti L, Scapozza L, et al. A novel FIP1L1-PDGFRA mutant destabilizing the inactive conformation of the kinase domain in chronic eosinophilic leukemia/hypereosinophilic syndrome. Allergy. 2009;64:913-8 pubmed publisher
    ..receptor alpha (FIP1L1-PDGFRA) gene fusion is a common cause of chronic eosinophilic leukemia (CEL)/hypereosinophilic syndrome (HES), and patients suffering from this particular subgroup of CEL/HES respond to low-dose imatinib ..
  24. Klion A. Hypereosinophilic syndrome: current approach to diagnosis and treatment. Annu Rev Med. 2009;60:293-306 pubmed publisher
    b>Hypereosinophilic syndrome is a heterogeneous group of rare disorders characterized by marked blood or tissue eosinophilia resulting in a wide variety of clinical manifestations...
  25. Rothenberg M, Klion A, Roufosse F, Kahn J, Weller P, Simon H, et al. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. N Engl J Med. 2008;358:1215-28 pubmed publisher
    The hypereosinophilic syndrome is a group of diseases characterized by persistent blood eosinophilia, defined as more than 1500 cells per microliter with end-organ involvement and no recognized secondary cause...
  26. Ishihara K, Kitamura H, Hiraizumi K, Kaneko M, Takahashi A, Zee O, et al. Mechanisms for the proliferation of eosinophilic leukemia cells by FIP1L1-PDGFRalpha. Biochem Biophys Res Commun. 2008;366:1007-11 pubmed
  27. Roufosse F, Cogan E, Goldman M. Lymphocytic variant hypereosinophilic syndromes. Immunol Allergy Clin North Am. 2007;27:389-413 pubmed
    ..evidence establishing the existence of an underlying T-cell disorder in a subset of patients fulfilling hypereosinophilic syndrome (HES) diagnostic criteria has accumulated over the past decade, resulting in the definition of a novel ..
  28. Nofal R. [Eosinophilic cystitis as symptom of an idiopathic hypereosinophilic syndrome]. Aktuelle Urol. 2007;38:148-51 pubmed
    Eosinophilic cystitis as a symptom of an idiopathic hypereosinophilic syndrome is a very rare clinical picture with an unpredictable course. A combination of both symptoms has not been described previously in the peer reviewed literature...
  29. Wicklein D, Ramos Leal N, Salamon J, Thamer M, Herrmann H, Valent P, et al. Nilotinib and imatinib are comparably effective in reducing growth of human eosinophil leukemia cells in a newly established xenograft model. PLoS ONE. 2012;7:e30567 pubmed publisher
    ..The new model system is feasible for the evaluation of new tyrosine kinase inhibitors and our data suggest that nilotinib may be a valuable additional targeted drug active in patients with FIP1L1/PDGFRA+ CEL...
  30. Vassina E, Yousefi S, Simon D, Zwicky C, Conus S, Simon H. cIAP-2 and survivin contribute to cytokine-mediated delayed eosinophil apoptosis. Eur J Immunol. 2006;36:1975-84 pubmed
    ..Here, we show that cultured eosinophils purified from blood of hypereosinophilic syndrome (HES) patients exhibit delayed spontaneous death and relative resistance towards ceramide- but not CD95-..
  31. Tefferi A, Patnaik M, Pardanani A. Eosinophilia: secondary, clonal and idiopathic. Br J Haematol. 2006;133:468-92 pubmed
    ..The current communication features a comprehensive clinical summary of both secondary and primary eosinophilic disorders with emphasis on recent developments in molecular pathogenesis and treatment...
  32. Lim J, Sternberg A, Manghat N, Ramcharitar S. Hypereosinophilic syndrome masquerading as a myocardial infarction causing decompensated heart failure. BMC Cardiovasc Disord. 2013;13:75 pubmed publisher
    An 81 year old female patient diagnosed with a chronic low grade hypereosinophilic syndrome presented with angina and dyspnoea.
  33. Simon H, Rothenberg M, Bochner B, Weller P, Wardlaw A, Wechsler M, et al. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol. 2010;126:45-9 pubmed publisher
    Because of advances in our understanding of the hypereosinophilic syndrome (HES) and the availability of novel therapeutic agents, the original criteria defining these disorders are becoming increasingly problematic...
  34. Elling C, Erben P, Walz C, Frickenhaus M, Schemionek M, Stehling M, et al. Novel imatinib-sensitive PDGFRA-activating point mutations in hypereosinophilic syndrome induce growth factor independence and leukemia-like disease. Blood. 2011;117:2935-43 pubmed publisher
    The FIP1L1-PDGFRA fusion is seen in a fraction of cases with a presumptive diagnosis of hypereosinophilic syndrome (HES)...
  35. Sheikh J, Weller P. Advances in diagnosis and treatment of eosinophilia. Curr Opin Hematol. 2009;16:3-8 pubmed publisher
    ..HES are now recognized to include varied eosinophilic disorders for some of which there are recent insights into their pathogenesis and targeted treatment...
  36. Helbig G, Moskwa A, Hus M, Piszcz J, Swiderska A, Urbanowicz A, et al. Clinical characteristics of patients with chronic eosinophilic leukaemia (CEL) harbouring FIP1L1-PDGFRA fusion transcript--results of Polish multicentre study. Hematol Oncol. 2010;28:93-7 pubmed publisher
    A small subgroup of patients with hypereosinophilic syndrome (HES) demonstrates imatinib-sensitive fusion transcript-the FIP1L1-PDGFRA (F/P+). These cases are currently diagnosed as chronic eosinophilic leukaemia (CEL)...
  37. Titlic M, Kodzoman K, Loncar D. Neurologic manifestations of hypereosinophilic syndrome--review of the literature. Acta Clin Croat. 2012;51:65-9 pubmed
    b>Hypereosinophilic syndrome is a rare disorder of the hematopoietic system. The disease is characterized by continuously high number of eosinophils (>1.5 x 10(9)/L) for more than six months...
  38. Terrier B, Piette A, Kerob D, Cordoliani F, Tancrède E, Hamidou L, et al. Superficial venous thrombophlebitis as the initial manifestation of hypereosinophilic syndrome: study of the first 3 cases. Arch Dermatol. 2006;142:1606-10 pubmed
    ..that can reveal neoplasia, malignant blood disorders, or vasculitis, but it has never been described in hypereosinophilic syndrome (HES)...
  39. Lierman E, Michaux L, Beullens E, Pierre P, Marynen P, Cools J, et al. FIP1L1-PDGFRalpha D842V, a novel panresistant mutant, emerging after treatment of FIP1L1-PDGFRalpha T674I eosinophilic leukemia with single agent sorafenib. Leukemia. 2009;23:845-51 pubmed publisher
    ..The identification of new PDGFR inhibitors will be required to overcome resistance by this D842V mutant...
  40. Lee J, Yang E, Kim I. The roles of MCP-1 and protein kinase C delta activation in human eosinophilic leukemia EoL-1 cells. Cytokine. 2009;48:186-95 pubmed publisher
    ..PKC delta functions as an anti-apoptotic molecule, and is involved in EoL-1 cell movement stimulated by MCP-1. This study contributes to an understanding of MCP-1 in eosinophil biology and pathogenic mechanism of eosinophilic disorders...
  41. Kahn J, Bletry O, Guillevin L. Hypereosinophilic syndromes. Best Pract Res Clin Rheumatol. 2008;22:863-82 pubmed publisher
    ..When a complete evaluation of a chronic hypereosinophilia fails to reveal an underlying disease, the diagnosis of hypereosinophilic syndrome (HES) or Churg-Strauss syndrome (CSS) is suggested.
  42. Buitenhuis M, Verhagen L, Cools J, Coffer P. Molecular mechanisms underlying FIP1L1-PDGFRA-mediated myeloproliferation. Cancer Res. 2007;67:3759-66 pubmed
  43. Sussman D, Bejarano P, Regev A. Eosinophilic cholangiopathy with concurrent eosinophilic colitis in a patient with idiopathic hypereosinophilic syndrome. Eur J Gastroenterol Hepatol. 2008;20:574-7 pubmed publisher
    ..We report a 52-year-old man with a history of idiopathic hypereosinophilic syndrome who presented with acute diarrhea and cholestatic hepatitis...
  44. Sato Y, Fukunaga T, Hayashi T, Asada Y. Hypereosinophilic syndrome associated with occlusive coronary thrombosis and right ventricular thrombus. Pathol Int. 2008;58:138-41 pubmed publisher
    ..The findings suggest a causal relationship between coronary thrombosis and eosinophilia. To the authors' knowledge this is the first report to document the clinical and histological findings of coronary thrombosis in a patient with HES...
  45. Ionescu M, Murata H, Janin A. Oral mucosa lesions in hypereosinophilic syndrome--an update. Oral Dis. 2008;14:115-22 pubmed publisher
    b>Hypereosinophilic syndrome (HES) is a rare disorder characterized by persistent and marked eosinophilia...
  46. Pitini V, Arrigo C, Altavilla G, Naro C, Righi M. Imatinib-induced apoptosis in the eosinophils of patients with a hypereosinophilic syndrome: a surrogate marker of response?. Leuk Res. 2007;31:725-6 pubmed
  47. Maino A, Rossio R, Cugno M, Marzano A, Tedeschi A. Hypereosinophilic syndrome, Churg-Strauss syndrome and parasitic diseases: possible links between eosinophilia and thrombosis. Curr Vasc Pharmacol. 2012;10:670-5 pubmed
    ..e. the hypereosinophilic syndrome (HES), the Churg Strauss syndrome (CSS), and parasitic infestations from 1966 to date...
  48. Ando J, Sugimoto K, Tamayose K, Ando M, Kojima Y, Oshimi K. Cytokine-producing sarcoma mimics eosinophilic leukaemia. Eur J Haematol. 2007;78:169-70 pubmed
  49. Kobayashi M, Komatsu N, Kuwayama Y, Bandobashi K, Kubota T, Uemura Y, et al. Idiopathic hypereosinophilic syndrome presenting acute abdomen. Intern Med. 2007;46:675-8 pubmed
    ..Molecular analysis demonstrated that the FIP1L1-PDGFRA fusion gene was negative. Histopathology showed thrombi and eosinophilic inflammation of arteries. It is important to recognize that HES could be a cause of acute abdomen...
  50. Metzgeroth G, Walz C, Erben P, Popp H, Schmitt Graeff A, Haferlach C, et al. Safety and efficacy of imatinib in chronic eosinophilic leukaemia and hypereosinophilic syndrome: a phase-II study. Br J Haematol. 2008;143:707-15 pubmed publisher
    ..study evaluated the efficacy and safety of imatinib in chronic eosinophilic leukaemia (CEL, n = 23) and hypereosinophilic syndrome (HES, n = 13)...
  51. Ogbogu P, Bochner B, Butterfield J, Gleich G, Huss Marp J, Kahn J, et al. Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy. J Allergy Clin Immunol. 2009;124:1319-25.e3 pubmed publisher
    b>Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders defined by persistent blood eosinophilia > or =1.5 x 10(9)/L, absence of a secondary cause, and evidence of eosinophil-associated pathology...
  52. Lee J, Kim I. Leukotactin-1/CCL15 induces cell migration and differentiation of human eosinophilic leukemia EoL-1 cells through PKCdelta activation. Mol Biol Rep. 2010;37:2149-56 pubmed publisher
    ..This study suggests the pivotal roles of Lkn-1 in the regulation of the movement and development of eosinophils...
  53. Kawata E, Kuroda J, Wada K, Yoshida M, Kamiuchi K, Nakayama Harusato I, et al. Hypereosinophilic syndrome accompanied by Buerger's disease-like femoral arterial occlusions. Intern Med. 2007;46:1919-22 pubmed
    A case of hypereosinophilic syndrome (HES) with a rare complication of Buerger's disease-like large arterial occlusion (AO) was successfully treated with emergent percutaneous transluminal angioplasty and anticoagulants plus ..
  54. Helbig G, Wiśniewska Piąty K, Francuz T, Dziaczkowska Suszek J, Kyrcz Krzemień S. Diversity of clinical manifestations and response to corticosteroids for idiopathic hypereosinophilic syndrome: retrospective study in 33 patients. Leuk Lymphoma. 2013;54:807-11 pubmed publisher
    Idiopathic hypereosinophilic syndrome (IHES) is characterized by blood hypereosinophilia with no underlying cause and eosinophilia-associated organ dysfunction...
  55. Helbig G, Stella Hołowiecka B, Majewski M, Całbecka M, Gajkowska J, Klimkiewicz R, et al. A single weekly dose of imatinib is sufficient to induce and maintain remission of chronic eosinophilic leukaemia in FIP1L1-PDGFRA-expressing patients. Br J Haematol. 2008;141:200-4 pubmed publisher
    b>Hypereosinophilic syndrome (HES) is defined as chronic, unexplained hypereosinophilia with organ involvement...
  56. Syed I, Martinez M, Feng D, Glockner J. Cardiac magnetic resonance imaging of eosinophilic endomyocardial disease. Int J Cardiol. 2008;126:e50-2 pubmed
    Idiopathic hypereosinophilic syndrome (HES) is rare and frequently involves the heart with development of endomyocardial fibrosis, a characteristic restrictive cardiomyopathy. Non-invasive diagnosis is frequently challenging...
  57. Klion A, Bochner B, Gleich G, Nutman T, Rothenberg M, Simon H, et al. Approaches to the treatment of hypereosinophilic syndromes: a workshop summary report. J Allergy Clin Immunol. 2006;117:1292-302 pubmed
    ..Switzerland to discuss current and future approaches to therapy for 3 eosinophil-mediated disorders: hypereosinophilic syndrome, Churg-Strauss syndrome, and eosinophil-associated gastrointestinal disease...
  58. Mehr S, Rego S, Kakakios A, Kilham H, Kemp A. Treatment of a case of pediatric hypereosinophilic syndrome with anti-interleukin-5. J Pediatr. 2009;155:289-91 pubmed publisher
    We report the use of anti-interleukin-5 (mepolizumab) during an 18-month period in a pediatric hypereosinophilic syndrome. Infusions every 3 months allowed better control of hypereosinophilic syndrome flares and maintained blood ..
  59. Benezet Mazuecos J, Marcos Alberca P, Farre J, Orejas M, de la Fuente A, Prieto E. Images in cardiovascular medicine. Early differential resolution of right and left ventricular obliteration in Löffler endocarditis after chemotherapy and anticoagulation. Circulation. 2006;114:e635-7 pubmed
  60. Score J, Curtis C, Waghorn K, Stalder M, Jotterand M, Grand F, et al. Identification of a novel imatinib responsive KIF5B-PDGFRA fusion gene following screening for PDGFRA overexpression in patients with hypereosinophilia. Leukemia. 2006;20:827-32 pubmed
    Idiopathic hypereosinophilic syndrome (IHES) is a disease that is difficult to classify, and diagnosis is one of exclusion...
  61. Gotlib J, Cross N, Gilliland D. Eosinophilic disorders: molecular pathogenesis, new classification, and modern therapy. Best Pract Res Clin Haematol. 2006;19:535-69 pubmed
    ..or bone marrow blasts) resulted in diagnosticians characterizing such nebulous cases as 'idiopathic hypereosinophilic syndrome (HES)'...
  62. Jovanovic J, Score J, Waghorn K, Cilloni D, Gottardi E, Metzgeroth G, et al. Low-dose imatinib mesylate leads to rapid induction of major molecular responses and achievement of complete molecular remission in FIP1L1-PDGFRA-positive chronic eosinophilic leukemia. Blood. 2007;109:4635-40 pubmed
  63. Burgstaller S, Kreil S, Waghorn K, Metzgeroth G, Preudhomme C, Zoi K, et al. The severity of FIP1L1-PDGFRA-positive chronic eosinophilic leukaemia is associated with polymorphic variation at the IL5RA locus. Leukemia. 2007;21:2428-32 pubmed
    ..001). These data suggest that the variations in IL5RA expression are linked to constitutional IL5RA genotype and severity of FIP1L1-PDGFRA disease...
  64. 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;15:368-73 pubmed publisher
    Patients with hypereosinophilic syndrome (HES) or chronic eosinophilic leukemia (CEL) that are refractory to standard therapies are difficult to manage and have significantly shortened life expectancy.
  65. Fukushima K, Matsumura I, Ezoe S, Tokunaga M, Yasumi M, Satoh Y, et al. FIP1L1-PDGFRalpha imposes eosinophil lineage commitment on hematopoietic stem/progenitor cells. J Biol Chem. 2009;284:7719-32 pubmed publisher
    ..Here we report the molecular mechanism underlying the development of hypereosinophilic syndrome/chronic eosinophilic leukemia by FIP1L1-PDGFRalpha...