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Klin Padiatr 2016; 228(06/07): 319-324
DOI: 10.1055/s-0042-112593
Originalarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Ein T-Zell assoziiertes Hypereosinophilie-Syndrom (L-HES) als Differenzialdiagnose eines Asthma bronchiale

The Lymphoid Variant of HES (L-HES) as Differential Diagnose of Severe Asthma in Childhood
T. Leu
1   Children’s Hospital, University of Würzburg, Würzburg
,
S. Rauthe
2   Institute of Pathology, University of Würzburg, Würzburg
,
C. Wirth
3   Institute of Radiology, University Hospital Würzburg, Würzburg
,
H.-U. Simon
4   Institute of pharmacology, University of Bern, Bern, Switzerland
,
V. Kunzmann
5   Division of Medical Oncology, Department of Internal Medicine II, Würzburg
,
H. Hebestreit
1   Children’s Hospital, University of Würzburg, Würzburg
,
S. Kunzmann
1   Children’s Hospital, University of Würzburg, Würzburg
6   Department of Neonatology and Pediatric Intensive Care Medicine, Bürgerhospital Frankfurt am Main
› Author Affiliations
Further Information

Publication History

Publication Date:
15 November 2016 (online)

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Zusammenfassung

Ein 13 Jahre alter Junge wurde zur Abklärung eines Asthma bronchiale aufgenommen. Im Blutbild fand sich eine HE von 3 500/µl (30%), zudem ein erhöhtes Gesamt-IgE von 2 000 IU/ml und ein erhöhter IL-5 Spiegel. In der Lungenfunktion konnte eine Obstruktion (FEV1 38%) nachgewiesen werden. Radiologisch zeigten sich Bronchiektasien und mucous pluggine. In der BAL fiel eine Eosinophilie (76%) mit erhöhtem IL-5 auf. Histologisch fand sich das Bild eines Asthma bronchiale mit einer Mukostase in Form von Curschmann-Spiralen, eine Hypertrophie der Bronchialwandmuskulatur sowie eine Lungeneosinophilie. Eine ABPA, ein Churg-Strauss-Syndrom, eine Parasitose, eine arzneimittelassoziierte HE, Allergien sowie eine maligne Grunderkrankung konnten ausgeschlossen werden. In der Klonalitäts-Analyse und der Durchflusszytometrie ergab sich der Nachweis einer klonalen T-Lymphozyten Population mit auffälligem Immunophänotyp, was zur Diagnose eines T-Zell assoziierten HES (L-HES) führte. Ein primär neoplastisches HES (HESN) konnte durch fehlenden Nachweis einer Fusion der FIP1L1- und PDGFRα Gene und eine unauffällige Knochenmarkspunktion ausgeschlossen werden. Eine Therapie mit Prednisolon führte zu einer raschen Besserung. Zur Glukokortikoideinsparung wurde eine immunsuppressive Therapie mit Azathioprin begonnen, worunter der Patient weitgehend beschwerdefrei blieb. Dieser Fall zeigt die Bandbreite in der HE Abklärung auf. Bei einem V. a. ein schweres Asthma bronchiale mit einer ausgeprägten HE sollte auch an die Möglichkeit eines L-HES gedacht werden.

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.

Schlüsselwörter

eosinophile Alveolitis - chronisch eosinophile Pneumonie - Azathioprin - Asthma

Key words

eosinophilic alveolitis - chronic eosinophilic pneumonia (CEP) - azathioprine - asthma
 

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