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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


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