title: [The Lymphoid Variant of HES (L-HES) as Differential Diagnose of Severe Asthma in Childhood].
abstract: Based on a case report an overview on the differential diagnostic considerations with respect to blood hypereosinophilia (HE) and hypereosinophilic syndromes (HES) in childhood is given. A 13-year-old boy was admitted for the clarification of an asthma. In the blood count an increased HE with 3 500/µl (30%) was found along with elevated total serum IgE and IL-5 level (2 000 IU/ml and 17 pg/ml). Lung function showed an obstruction (FEV1 38%). Radiologically the picture of bronchiectasis and mucus pluggine appeared. In the BAL a HE (76%) with raised IL-5 level was apparent. Histologically asthma was diagnosed with mucostasis, hypertrophy of the bronchial wall musculature and a lung HE. Differential-diagnostically an ABPA, a Churg-Strauss-Syndrome, a parasitosis, drug associated HE, allergies and malignant disease could be excluded. An aberrant T-cell clone in peripheral blood was detected by flow cytometry and T-cell receptor clonal rearrangements by PCR, leading to the diagnosis of a lymphoid variant of HES (L-HES). Failure to detect the FIP1L1-PDGFRA gene fusion and a normal bone marrow examination could exclude a neoplastic HES (HESN). After steroid initiation, prompt decrease of blood eosinophilia with resolution of symptoms was observed. Steroid discontinuation led to eosinophilia recurrence associated with disease symptoms. As steroid-sparing agent the immunosuppressive azathioprine was additionally given; steroid doses could be decreased and stopped in the course. This case demonstrated the range of HE evaluation in infancy. With asthma one should also consider the possibility of a L-HES.
authors.affiliation: Children's Hospital, University of Würzburg, Würzburg.
authors.forename: T
authors.initials: T
authors.lastname: Leu
authors.affiliation: Institute of Pathology, University of Würzburg, Würzburg.
authors.forename: S
authors.initials: S
authors.lastname: Rauthe
authors.affiliation: Institute of Radiology, University Hospital Würzburg, Würzburg.
authors.forename: C
authors.initials: C
authors.lastname: Wirth
authors.affiliation: Institute of pharmacology, University of Bern, Bern, Switzerland.
authors.forename: H-U
authors.initials: HU
authors.lastname: Simon
authors.affiliation: Division of Medical Oncology, Department of Internal Medicine II, Würzburg.
authors.forename: V
authors.initials: V
authors.lastname: Kunzmann
authors.affiliation: Children's Hospital, University of Würzburg, Würzburg.
authors.forename: H
authors.initials: H
authors.lastname: Hebestreit
authors.affiliation: Children's Hospital, University of Würzburg, Würzburg.
authors.forename: S
authors.initials: S
authors.lastname: Kunzmann
mesh_terms: D000293:Adolescent; D001249:Asthma / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D001379:Azathioprine / Q000627:therapeutic use; D001707:Biopsy, Needle; D001853:Bone Marrow / Q000473:pathology; D001980:Bronchi / Q000473:pathology; D003937:Diagnosis, Differential; D005434:Flow Cytometry; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D007073:Immunoglobulin E / Q000097:blood; D015848:Interleukin-5 / Q000097:blood; D008168:Lung / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D011657:Pulmonary Eosinophilia / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000473:pathology; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics; D013601:T-Lymphocytes / Q000276:immunology*; D039221:mRNA Cleavage and Polyadenylation Factors / Q000235:genetics
chemical_list: D015848:Interleukin-5; D015514:Oncogene Proteins, Fusion; D039221:mRNA Cleavage and Polyadenylation Factors; D007073:Immunoglobulin E; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha; D001379:Azathioprine