Subtype classification of hypereosinophilic syndromes (HES).
| *antibodies specific for the following additional markers should be included if routine phenotyping is normal and the lymphoproliferative variant is suspected: CD2, CD3, CD4, CD5, CD6, CD7, CD8, CD25, CD27, CD45RO, TCRα/β, TCRγ/δ, HLA-DR and CD95 |
| ‡ TARC-CC thymus and activation related chemokine (CCL17) |
| Myeloproliferative variant |
| Definitive Evidence |
| FIP1L1-PDGFRA fusion by RT-PCR or FISH |
| Eosinophil clonality by HUMARA analysis, karyotype or other modality |
| Supportive Evidence |
| ≥ 4 of the following: |
| increased serum tryptase level |
| increased serum B12 level |
| splenomegaly |
| anemia, thrombocytopenia |
| increased circulating myeloid precursors |
| dysplastic eosinophils |
| myelofibrosis |
| increased spindle-shaped mast cells in the bone marrow |
| Lymphoproliferative variant |
| Definitive Evidence |
| Phenotypically aberrant T cell population* |
| Clonal T cell rearrangement pattern by PCR |
| Increased T cell production of eosinophilopoietic cytokines |
| Supportive Evidence |
| Increased serum TARC‡ |
| Increased serum IgE |
| Predominantly cutaneous manifestations |
| History of atopy |
| Steroid-responsive |
| *antibodies specific for the following additional markers should be included if routine phenotyping is normal and the lymphoproliferative variant is suspected: CD2, CD3, CD4, CD5, CD6, CD7, CD8, CD25, CD27, CD45RO, TCRα/β, TCRγ/δ, HLA-DR and CD95 |
| ‡ TARC-CC thymus and activation related chemokine (CCL17) |
| Myeloproliferative variant |
| Definitive Evidence |
| FIP1L1-PDGFRA fusion by RT-PCR or FISH |
| Eosinophil clonality by HUMARA analysis, karyotype or other modality |
| Supportive Evidence |
| ≥ 4 of the following: |
| increased serum tryptase level |
| increased serum B12 level |
| splenomegaly |
| anemia, thrombocytopenia |
| increased circulating myeloid precursors |
| dysplastic eosinophils |
| myelofibrosis |
| increased spindle-shaped mast cells in the bone marrow |
| Lymphoproliferative variant |
| Definitive Evidence |
| Phenotypically aberrant T cell population* |
| Clonal T cell rearrangement pattern by PCR |
| Increased T cell production of eosinophilopoietic cytokines |
| Supportive Evidence |
| Increased serum TARC‡ |
| Increased serum IgE |
| Predominantly cutaneous manifestations |
| History of atopy |
| Steroid-responsive |