Клональні гіпереозинофільні синдроми
Feature | Myeloproliferative Variant | Lymphoproliferative Variant |
|---|---|---|
Genetics | Rearrangement of PDGFRA, PDGFRB, FGFR1, PCM1-JAK2, or ETV6-ABL1 | Clonal population of T cells with aberrant phenotype |
Clinical manifestations and laboratory findings | Anemia Elevated serum tryptase levels Elevated serum vitamin B12 levels Endomyocardial fibrosis Hypogranular or vacuolated eosinophils Myelofibrosis Splenomegaly Thrombocytopenia | Angioedema Circulating immune complexes (sometimes with serum sickness) Hypergammaglobulinemia (especially IgE) Skin abnormalities |
Increased risk of future disorder | T-cell lymphoma | |
Responsiveness to medications | Imatinib and other tyrosine kinase inhibitors | Corticosteroids |
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