The idiopathic hypereosinophilic syndrome

Blood eosinophilia signifies either a cytokine-mediated reactive phenomenon (secondary) or an integral phenotype of an underlying haematological neoplasm (primary) Secondary eosinophilia is usually associated with parasitosis in Third World countries and allergic conditions in the West Primary eosinophilia is operationally classified as being clonal or idiopathic, depending on the respective presence or absence of a molecular, cytogenetic or histological evidence for a myeloid malignancy The current communication features a comprehensive clinical summary of both secondary and primary eosinophilic disorders with emphasis on recent developments in molecular pathogenesis and treatment

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1365-2141.2006.06038.x

Eosinophilia: secondary, clonal and idiopathic.

An elevated blood eosinophil count may be associated with a number of reactive conditions and with clonal disorders of the bone marrow. When the blood eosinophil count is persistently greater than 1Æ5 · 10 ⁄ l for a period of 6 months, and damage to end organs such as the heart, lungs, skin, joints and nervous system can be demonstrated then, in the absence of any clonal abnormality or reactive cause, the term idiopathic hypereosinophilic syndrome (HES) is used. There is evidence to suggest that, in some patients, clonal T-cell populations are present. Treatment of the idiopathic hypereosinophilic syndrome has been varied and has included steroids, hydroxyurea, interferon and Tcell agents, and chemotherapy. Recently, patients have been treated successfully with imatinib mesylate and a trial of its use in HES is planned.

https://onlinelibrary.wiley.com/doi/pdf/10.1046/j.1365-2141.2003.04195.x

The eosinophilias, including the idiopathic hypereosinophilic syndrome.

Use of the term "idiopathic hypereosinophilic syndrome (HES)" has highlighted our basic lack of understanding of the molecular pathophysiology of eosinophilic disorders. However, over the last 10 years, the study of hypereosinophilia has enjoyed a revival. This interest has been rekindled by two factors: (1) the development of increasingly sophisticated molecular biology techniques that have unmasked recurrent genetic abnormalities linked to eosinophilia, and (2) the successful application of targeted therapy with agents such as imatinib to treat eosinophilic diseases. To date, most of these recurrent molecular abnormalities have resulted in constitutively activated fusion tyrosine kinases whose phenotypic consequence is an eosinophilia-associated myeloid disorder. Most notable among these are rearrangements of platelet-derived growth factor receptors alpha and beta (PDGFRalpha, PDGFRbeta), which define a small subset of patients with eosinophilic chronic myeloproliferative disorders (MPDs) and/or overlap myelodysplastic syndrome/MPD syndromes, including chronic myelomonocytic leukemia. Discovery of the cryptic FIP1L1-PDGFRA gene fusion in cytogenetically normal patients with systemic mast cell disease with eosinophilia or idiopathic HES has redefined these diseases as clonal eosinophilias. A growing list of fibroblast growth factor receptor 1 fusion partners has similarly emerged in the 8p11 myeloproliferative syndromes, which are often characterized by elevated eosinophil counts. Herein the focus is on the molecular gains made in these MPD-type eosinophilias, and the classification and clinicopathological issues related to hypereosinophilic syndromes, including the lymphocyte variant. Success in establishing the molecular basis of a group of once seemingly heterogeneous diseases has now the laid the foundation for establishing a semi-molecular classification scheme of eosinophilic disorders.

Molecular classification and pathogenesis of eosinophilic disorders: 2005 update.

A 49-year-old man with the idiopathic hypereosinophilic syndrome (HES) and a unique chromosomal abnormality 46,XY,t(5;9)(q32;q33) is reported. Complete cytogenetic remission was induced by interferon alpha-2b (IFN-alpha). The beneficial action of IFN-alpha in different stem-cell disorders such as CML, HES, multiple myeloma and solid tumours such as hypernephroma or malignant melanoma suggests a common regulatory effect possibly by immunomodulation or other (immune-mediated) mechanisms, but the exact pathophysiological mechanisms remain hypothetic and unresolved. Since it has been known for some years that the genes encoding for GM-CSF, IL-3 and IL-5 reside on the long arm of chromosome 5, it could be possible that the chromosomal translocation in our patient resulted in excess production of these cytokines, hence causing the hypereosinophilia. This case report and the results obtained from the literature review support the growing body of evidence that IFN-alpha has a major place in the long-term treatment of HES, especially in those cases resistant to conventional treatment, with cytogenetic abnormalities, or presenting as a myeloproliferative variant of HES. In those cases IFN-alpha results in lower morbidity, lower mortality and long-term survival.

Further evidence for the clonal nature of the idiopathic hypereosinophilic syndrome: complete haematological and cytogenetic remission induced by interferon-alpha in a case with a unique chromosomal abnormality

A unique chromosomal abnormality in idiopathic hypereosinophilic syndrome presenting with cardiac involvement.

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A unique chromosomal abnormality in idiopathic hypereosinophilic syndrome presenting with cardiac involvement.