Version October 2024
| Terminology for HES variants | Subtypes/identified abnormalities/examples | Clinical and laboratory features |
| Myeloid variants | PDGFRB and FGFR1 rearrangements JAK2 point mutation and translocation Chronic eosinophilic leukemia, not otherwise specified, on the basis of blast count in peripheral blood and/or bone marrow or of a clonal cytogenetic abnormality | ↑ Serum B12 Anemia and/or thrombocytopenia Hepatomegaly and/or splenomegaly Circulating leukocyte precursors |
| Deletion on 4q12 → FIP1L1-PDGFRA fusion | May show ↑ serum tryptase + mast cell abnormalities | |
| PDGFRA or PDGFRB rearrangement-associated disease occurs almost exclusively in males | ||
| T cell lymphocytic variants (L-HES) | Aberrant IL-5-producing T cells Example: CD3-CD4+ T cell-associated disease | Prominent skin findings (including plaques, erythroderma, urticaria) Polyclonal hypergammaglobulinemia Usually a benign lymphoproliferative disorder, but may progress to T cell lymphoma |
| Familial HES | Mapped to 5q 31-33 | Asymptomatic eosinophilia from birth, autosomal dominant Progression to other forms of HES may occur |
| Idiopathic HES | Multisystem involvement with varied signs/symptoms | |
| Organ-restricted HES | Examples include eosinophilic gastrointestinal disease, chronic eosinophilic pneumonia, and others | Peripheral blood eosinophilia associated with eosinophilic infiltration and associated signs/symptoms in a single organ |
| Specific/defined syndromes associated with hypereosinophilia | Examples include episodic angioedema with eosinophilia, eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss), other disorders associated with immune dysregulation | Marked eosinophilia in the setting of an underlying disorder associated with eosinophilia; the precise role of eosinophils in the disease manifestations remains uncertain |
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