[The Lymphoid Variant of HES (L-HES) as Differential Diagnose of Severe Asthma in Childhood].

Leu, T; Rauthe, S; Wirth, C; Simon, H-U; Kunzmann, V; Hebestreit, H; Kunzmann, S; Simon, Hans-Uwe (2016). [The Lymphoid Variant of HES (L-HES) as Differential Diagnose of Severe Asthma in Childhood]. Klinische Pädiatrie, 228(06/07), pp. 319-324. Thieme 10.1055/s-0042-112593

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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.

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