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After exclusion of secondary causes of eosinophilia, diagnostic evaluation of primary eosinophilias relies on a combination of morphologic review of the blood and marrow, standard cytogenetics, fluorescent in situ hybridization, flow immunocytometry, and T\u2010cell clonality assessment to detect histopathologic or clonal evidence for an acute or chronic myeloid or lymphoproliferative disorder.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p><jats:bold><jats:italic>Risk stratification<\/jats:italic><\/jats:bold>: Disease prognosis relies on identifying the subtype of eosinophilia. After evaluation of secondary causes of eosinophilia, the 2008 World Health Organization establishes a semimolecular classification scheme of disease subtypes including \u201cmyeloid and lymphoid neoplasms with eosinophilia and abnormalities of <jats:italic>PDGFRA<\/jats:italic>, <jats:italic>PDGFRB<\/jats:italic>, or <jats:italic>FGFR1<\/jats:italic>', chronic eosinophilic leukemia, not otherwise specified\u201d (CEL, NOS), lymphocyte\u2010variant HE, and idiopathic hypereosinophilic syndrome (HES), which is a diagnosis of exclusion.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p><jats:bold><jats:italic>Risk\u2010adapted therapy<\/jats:italic><\/jats:bold>: The goal of therapy is to mitigate eosinophil\u2010mediated organ damage. For patients with milder forms of eosinophilia (e.g., &lt;1,500\/mm<jats:sup>3<\/jats:sup>) without symptoms or signs of organ involvement, a watch and wait approach with close\u2010follow\u2010up may be undertaken. Identification of rearranged <jats:italic>PDGFRA<\/jats:italic> or <jats:italic>PDGFRB<\/jats:italic> is critical because of the exquisite responsiveness of these diseases to imatinib. Corticosteroids are first\u2010line therapy for patients with lymphocyte\u2010variant HE and HES. Hydroxyurea and interferon\u2010alpha have demonstrated efficacy as initial treatment and steroid\u2010refractory cases of HES. In addition to hydroxyurea, second\u2010line cytotoxic chemotherapy agents and hematopoietic cell transplant have been used for aggressive forms of HES and CEL with outcomes reported for limited number of patients. Although clinical trials have been performed with anti\u2010IL\u20105 (mepolizumab) and anti\u2010CD52 (alemtuzumab) antibodies, their therapeutic role in primary eosinophilic diseases and HES has yet to be established. Am. J. Hematol. 89:326\u2013337, 2014. \u00a9 2014 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajh.23664","type":"journal-article","created":{"date-parts":[[2014,2,28]],"date-time":"2014-02-28T04:37:40Z","timestamp":1393562260000},"page":"325-337","source":"Crossref","is-referenced-by-count":115,"title":["World Health Organization\u2010defined eosinophilic disorders: 2014 update on diagnosis, risk stratification, and management"],"prefix":"10.1002","volume":"89","author":[{"given":"Jason","family":"Gotlib","sequence":"first","affiliation":[{"name":"Division of Hematology Stanford Cancer Center Stanford California"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,2,27]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2010.03.035"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2008.287"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-10-050054"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-03-0787"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.leukres.2005.11.011"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(87)80352-9"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-200301000-00017"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mph.0000193467.06938.77"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa025217"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.11420"},{"key":"e_1_2_7_12_1","first-page":"963","article-title":"Eosinophilia detected by automated blood cell counting in ambulatory North American outpatients. 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