Table 3 The main subtypes of primary (neoplastic) hypereosinophilic syndromes
Subtype | Clinical features | Laboratory tests |
|---|---|---|
Myeloproliferative (M-HES) | Male predominance Hepato-splenomegaly Anemia Endomyocardial fibrosis Restrictive lung disease Mucosal ulcerations Good response to imatinib Variable steroid response Poor prognosis | F/P gene mutation by RT-PCR or FISH Increased serum tryptase Increased serum B12 Thrombocytopenia Dysplastic eosinophils Myelofibrosis Myeloid precursors in blood |
Lymphocytic (L-HES) | Almost equal sex ratio History of atopy Frequent skin lesions Gastrointestinal symptoms Obstructive lung disease Low mortality Possible progression to T-cell lymphoma Rare cardiac involvement Steroid responsive | Aberrant phenotypic T-cell population in blood Clonal T-cell pattern by PCR Increased eosinophilopoietic cytokines (IL-5) Increased serum IgE Increased TARC |