Table 1.

Disorders associated with marked eosinophilia

CategoryExamplesComments
Atopic disorders Asthma; atopic dermatitis; chronic rhinosinusitis Typically cause mild to moderate eosinophilia 
Drug hypersensitivity Varied; drug rash, eosinophilia, and systemic symptoms; eosinophilia-myalgia syndrome Can occur with any drug or supplement; manifestations range from asymptomatic eosinophilia to life-threatening complications40  
Infection and infestation Helminth infection (especially those with a tissue invasive phase)
Fungal infections
Viral infection (HIV, COVID-19)
Ectoparasite infestation
Protozoal infection (limited to Sarcocystis and Cystoisospora)
Tuberculosis (rare) 
Most common etiology worldwide; Strongyloides infection should always be considered due to worldwide distribution and the potential for fatal dissemination with steroid therapy41  
Autoimmune and immunodysregulatory disorders Inflammatory bowel disease, sarcoidosis, IgG4 disease Clinical sequelae of eosinophilia may or may not be present and can be difficult to distinguish from the manifestations of the underlying disorder 
Neoplasia Leukemia, lymphoma, solid tumors (especially adenocarcinoma) Although any leukemia/lymphoma can present with HE/HES, pre–B-cell acute lymphoblastic leukemia can be particularly difficult to diagnose 
Inborn errors of immunity Omenn syndrome, DOCK8 deficiency, Loeys-Dietz syndrome Usually diagnosed in childhood, recurrent or unusual infections common42  
Rare hypereosinophilic syndromes Eosinophilic myeloid neoplasms, lymphocytic variant HE/HES, idiopathic HE/HES, familial HE/HES, single-organ HE/HES, and other overlap disorders See Figure 2 for additional details 
Other Radiation, hypoadrenalism, cholesterol emboli, administration of IL-2  
CategoryExamplesComments
Atopic disorders Asthma; atopic dermatitis; chronic rhinosinusitis Typically cause mild to moderate eosinophilia 
Drug hypersensitivity Varied; drug rash, eosinophilia, and systemic symptoms; eosinophilia-myalgia syndrome Can occur with any drug or supplement; manifestations range from asymptomatic eosinophilia to life-threatening complications40  
Infection and infestation Helminth infection (especially those with a tissue invasive phase)
Fungal infections
Viral infection (HIV, COVID-19)
Ectoparasite infestation
Protozoal infection (limited to Sarcocystis and Cystoisospora)
Tuberculosis (rare) 
Most common etiology worldwide; Strongyloides infection should always be considered due to worldwide distribution and the potential for fatal dissemination with steroid therapy41  
Autoimmune and immunodysregulatory disorders Inflammatory bowel disease, sarcoidosis, IgG4 disease Clinical sequelae of eosinophilia may or may not be present and can be difficult to distinguish from the manifestations of the underlying disorder 
Neoplasia Leukemia, lymphoma, solid tumors (especially adenocarcinoma) Although any leukemia/lymphoma can present with HE/HES, pre–B-cell acute lymphoblastic leukemia can be particularly difficult to diagnose 
Inborn errors of immunity Omenn syndrome, DOCK8 deficiency, Loeys-Dietz syndrome Usually diagnosed in childhood, recurrent or unusual infections common42  
Rare hypereosinophilic syndromes Eosinophilic myeloid neoplasms, lymphocytic variant HE/HES, idiopathic HE/HES, familial HE/HES, single-organ HE/HES, and other overlap disorders See Figure 2 for additional details 
Other Radiation, hypoadrenalism, cholesterol emboli, administration of IL-2  

COVID-19, coronavirus disease 2019; HIV, human immunodeficiency virus.

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