Table 2.
Variant of HE | Abbreviation | Features |
---|---|---|
| ||
Hereditary (Familial) HE | HEFA | Familial clustering, often evidence of a hereditary immunodeficiency (inborn (errors of immunity with eosinophilia), no evidence of a reactive or neoplastic underlying disease, and no signs or symptoms indicative of HES |
HE of Unknown Significance | HEUS | No known underlying etiology of HE, no positive family history, no evidence of a reactive or neoplastic condition or disorder underlying HE, and no signs or symptoms indicative of HES |
Secondary (Reactive) HE | HER | Underlying reactive condition or disease that explains HE, no evidence for a clonal bone marrow disease that explains HE*; and no signs or symptoms indicative of HES |
Clonal (Neoplastic) HE | HEN | Underlying stem cell, myeloid, or eosinophil neoplasm inducing HE*; no signs/symptoms indicative of HES |
In clonal/neoplastic HE (HEN), eosinophils are considered to be clonal cells derived from neoplastic stem cells, whereas in reactive HE (HER), eosinophils are considered to be reactive (non-clonal) cells triggered by eosinopoietic cytokines such as interleukin-5. Abbreviations: HE, hypereosinophilia; HES, hypereosinophilic syndrome(s).