The Hypereosinophilic Syndromes in Childhood.

Leu, T; Simon, Hans-Uwe; Hebestreit, H; Kunzmann, S (2015). The Hypereosinophilic Syndromes in Childhood. Klinische Pädiatrie, 227(6-07), pp. 308-313. Thieme 10.1055/s-0035-1555792

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The hypereosinophilic syndromes are rare disorders in childhood and require extensive differential diagnostic considerations. In the last years the earlier "idiopathic HES" called syndromes could be differentiated into molecular biologically, immunophenotypically and clinically more characterized heterogeneous diseases with high therapeutic and prognostic relevance. Nowadays the term HES summarizes diseases, which go hand in hand with a local or systemic hypereosinophilia (HE) connected with an organ damage. Depending on the cause of the HE one differentiates primary/neoplastic HES (HESN) from secondary/reactive HES (HESR). The latter develops reactively in connection with allergies, parasitosis, medications, neoplasia or a clonal increase of T-lymphocytes among others. With HESN the HE results from a clonal increase of eosinophilic granulocytes. While for some subgroups of the HESN (among others FIP1L1-PDGFRA fusion gene) the administration of a tyrosine kinase inhibitor is a new and effective therapy option, glucocorticoids still represent the medication of first choice for many not PDGFRA associated variants. Different immunomodulatory drugs or cytostatic agents are necessary to allow dose reduction of glucocorticoids. The promising therapy with anti-IL-5 antibodies is still not approved in infancy, could however become a treatment option in the future. Due to the present lack of knowledge about the HES in infancy the establishment of a register should be aimed for the treatment of HES in infancy.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Pharmacology

UniBE Contributor:

Simon, Hans-Uwe

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1439-3824

Publisher:

Thieme

Language:

English

Submitter:

Debora Scherrer

Date Deposited:

11 Sep 2015 10:39

Last Modified:

19 Feb 2019 15:58

Publisher DOI:

10.1055/s-0035-1555792

PubMed ID:

26267413

BORIS DOI:

10.7892/boris.71498

URI:

https://boris.unibe.ch/id/eprint/71498

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