HES (Hypereosinophilic Syndrome)

EOS.JPG

Dr. Zhou presented an interesting case of a young previously healthy female with sub-acute relapsing periarticular edema and hypereosinophilia, ultimately felt to be secondary to lymphocytic variant of HES.

She shared her ddx for eosinophilia (CHINAA)

C – connective tissue diseases, e.g. eGPA, GPA, RA, dermatomyositis, scleroderma, Sjogren’s, SLE, Behcet’s IgG-related disease, APLS, immunodeficiencies

H – helminths

I – idiopathic (HES)

N – neoplasms, e.g. AEL, CEL, CML, systemic mastocytosis, B cell lymphoma, T cell lymphomas/leukemias, solid tumors (esp. GI tract, lungs, SCCs)

A – allergies, e.g. asthma, DRESS, ABPA

A – adrenal insufficiency

HES: AEC >1500 on 2 occasions + evidence of organ dysfunction

Primary HES: Due to neoplasm/clonal expansion

Secondary: Due to other etiologies (noted above, with helminth infections being the most common) , certain solid tumors, and T-cell lymphoma. Lymphocytic variant HES is a sub-variant in this category.


Patients with primary eosinophil counts > 100K/or rapidly rising eosinophil counts should be urgently referred to hematology and started on empiric treatment (pulse dose steroids + ivermectin if strongyloides positive).