Hypereosinophilic syndrome (HES) or idiopathic eosinophilia syndrome

Abstract:
The idiopathic eosinophilia syndromes are a heterogeneous group of diseases which are characterized by a persitent eosinophilia > 1.5 x 109/L for more than six months with tissue lesions. The diagnosis is established after excluding other causes of an eosinophilia.

Clinical picture:
Hypereosinophilic syndromes can manifest with urticaria, fever, cough, shortness of breath, diarrhea and peripheral neuropathy. Tissue infiltration can lead to chronic congestive cardiomyopathy with valvular change and thickening of the endocardium with mural thrombi.

Hematology:
Eosinophils look normal for the most part. Often, however, they are also hypogranular and vacuolated with hyposegmented or hypersegmented nuclei. Additionally, anemia, anisocytosis, poikilocytosis, basophilia, thrombocytopenia or thrombocytosis as well as neutrophilia can occur.

Bone marrow:
Cellularity of the bone marrow is usually increased. The eosinophilic elements constitute between 25 and 75% of the cells.


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