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TableĀ 3 The main subtypes of primary (neoplastic) hypereosinophilic syndromes

From: Eosinophilic disorders: evaluation of current classification and diagnostic criteria, proposal of a practical diagnostic algorithm

Subtype Clinical features Laboratory tests
Myeloproliferative (M-HES) Male predominance
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
Dysplastic eosinophils
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
  1. TARC thymus activation-regulated chemokine, F/P mutation FIP1L1-PDGFRA mutation, FISH fluorescence in situ hybridization, RT-PCR reverse transcriptase polymerase chain reaction