Hypereosinophilic syndromes - An enigmatic group of disorders with an intriguing clinical spectrum and challenging treatment
Copyright © 2021 Elsevier Ltd. All rights reserved..
Hypereosinophilic syndromes (HES) comprises a group of rare disorders characterized by blood hypereosinophilia (>1.5 × 109/l) accompanied by eosinophil-associated organ damage. The 2016 World Health Organization classification recognizes a category of myeloid/lymphoid neoplasms with prominent eosinophilia (M/Leo) and well-characterized gene rearrangements of PDGFRA/B, FGFR1 or JAK2. PDGFRA/B-rearranged patients usually manifest as imatinib-sensitive myeloproliferative neoplasms (MPNs). FGFR1- and JAK2- rearranged cases may manifest as MPNs or aggressive lymphomas/leukemias and historically have had a dismal prognosis, although clinical trials with targeted treatment are promising. A negative screen for M/Leo in a patient with myeloid features should prompt consideration of a diagnosis of chronic eosinophilic leukemia-not otherwise specified. If these are excluded and a secondary cause is not identified, a diagnosis of idiopathic HES and/or other rare variants of HES should be considered. This review, through an illustrative case, summarizes current knowledge on HES pointing at new directions in diagnosis and treatment.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:49 |
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| Enthalten in: |
Blood reviews - 49(2021) vom: 10. Sept., Seite 100809 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Helbig, Grzegorz [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 17.11.2021 Date Revised 17.11.2021 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.blre.2021.100809 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| 520 | |a Hypereosinophilic syndromes (HES) comprises a group of rare disorders characterized by blood hypereosinophilia (>1.5 × 109/l) accompanied by eosinophil-associated organ damage. The 2016 World Health Organization classification recognizes a category of myeloid/lymphoid neoplasms with prominent eosinophilia (M/Leo) and well-characterized gene rearrangements of PDGFRA/B, FGFR1 or JAK2. PDGFRA/B-rearranged patients usually manifest as imatinib-sensitive myeloproliferative neoplasms (MPNs). FGFR1- and JAK2- rearranged cases may manifest as MPNs or aggressive lymphomas/leukemias and historically have had a dismal prognosis, although clinical trials with targeted treatment are promising. A negative screen for M/Leo in a patient with myeloid features should prompt consideration of a diagnosis of chronic eosinophilic leukemia-not otherwise specified. If these are excluded and a secondary cause is not identified, a diagnosis of idiopathic HES and/or other rare variants of HES should be considered. This review, through an illustrative case, summarizes current knowledge on HES pointing at new directions in diagnosis and treatment | ||
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| 650 | 4 | |a Journal Article | |
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| 650 | 4 | |a Hypereosinophilic syndromes | |
| 650 | 4 | |a Outcome | |
| 650 | 4 | |a Pathogenesis | |
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