[The Hypereosinophilic Syndromes in Childhood].
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.
Universitäts-Kinderklinik Würzburg, Würzburg.
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Leu
Institut für Pharmakologie, Universität Bern, Bern.
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HU
Simon
Universitäts-Kinderklinik Würzburg, Würzburg.
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Hebestreit
Universitäts-Kinderklinik Würzburg, Würzburg.
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Kunzmann
D061067:Antibodies, Monoclonal, Humanized / Q000009:adverse effects / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D054697:Cytostatic Agents / Q000627:therapeutic use; D003937:Diagnosis, Differential; D017277:Drug Approval; D005858:Germany; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D000068877:Imatinib Mesylate / Q000009:adverse effects / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007223:Infant; D015848:Interleukin-5 / Q000037:antagonists & inhibitors; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D035583:Rare Diseases
D061067:Antibodies, Monoclonal, Humanized; D054697:Cytostatic Agents; D005938:Glucocorticoids; D007155:Immunologic Factors; D015848:Interleukin-5; D000068877:Imatinib Mesylate; C434107:mepolizumab; D011505:Protein-Tyrosine Kinases