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Klin Padiatr 2015; 227(06/07): 308-313
DOI: 10.1055/s-0035-1555792
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Die Hypereosinophilie-Syndrome (HES) im Kindesalter

The Hypereosinophilic Syndromes in Childhood
T. Leu
1   Universitäts-Kinderklinik Würzburg, Würzburg
3   Kinderklinik, Klinikum Nürnberg-Süd, Nürnberg
,
H.-U. Simon
2   Institut für Pharmakologie, Universität Bern, Bern
,
H. Hebestreit
1   Universitäts-Kinderklinik Würzburg, Würzburg
,
S. Kunzmann
1   Universitäts-Kinderklinik Würzburg, Würzburg
› Author Affiliations
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Publication History

Publication Date:
12 August 2015 (online)

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Zusammenfassung

Die Hypereosinophilie-Syndrome (HES) sind in der Pädiatrie nur selten anzutreffende Erkrankungen und erfordern umfangreiche differenzialdiagnostische Überlegungen. In den letzten Jahren konnte das früher als „idiopathische HES“ bezeichnete Krankheitsbild mehr und mehr in molekularbiologisch, immunphänotypisch und klinisch näher charakterisierte heterogene Krankheitsbilder mit hoher therapeutischer und prognostischer Relevanz differenziert werden. Unter dem Begriff HES werden heute Erkrankungen zusammengefasst, die mit einer systemischen oder lokalen Hypereosinophilie (HE) und einer damit verbunden Schädigung der Organe einhergehen. In Abhängigkeit von der Ursache unterscheidet man primär-neoplastische HES (HESN) und sekundär/reaktive HES (HESR). Letztere entstehen reaktiv u. a. in Zusammenhang mit einer klonalen Vermehrung von T-Lymphozyten, Allergien, Parasitosen, Medikamenteneinnahmen oder Neoplasien. Bei HESN entsteht die HE durch eine klonale Vermehrung der Eosinophilen. Während für einige Subgruppen der HESN (u. a. FIP1L1-PDGFRA-Fusionsgen) die Gabe von Tyrosinkinase-Inhibitoren eine neue und effektive Therapieoption darstellt, sind Glukokortikoide weiterhin für viele nicht PDGFRA assoziierte Varianten das Präparat der ersten Wahl. Zur oftmals nötigen Dosiseinsparung der Glukokortikoide kommen verschiedene immunmodulatorische oder zytostatische Medikamente zum Einsatz. Die vielversprechende Therapie mit Anti-IL-5 Antikörpern besitzt derzeit (noch) keine Zulassung im Kindesalter, könnte aber für die Zukunft eine Behandlungsoption werden. Aufgrund des derzeitigen Mangels an Wissen über die HES im Kindesalter sollte die Einrichtung eines Registers zur Behandlung der HES im Kindesalter angestrebt werden.

Abstract

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.

Schlüsselwörter

eosinophile Alveolitis - Asthma bronchiale - Churg-Strauss-Syndrom - Imatinib - Mepolizumab

Key words

eosinophilic alveoltitis - asthma - churg-strauss-syndrome - imatinib - mepolizumab
 

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