Syndrome HES

• Must exclude infectious, inflammatory and neoplastic causes of eosinophilia including CML, AML with inv(16), other chronic myeloproliferative disorders, lymphoma (esp. Hodgkin's).

• Evidence of clonality = CeL; no evidence of clonality = HES.

• CEL is uncommon; marked increase in mature and immature eosinophils in PB and BM. Often >5% blasts in BM. Associated with tissue infiltration by immature eosinophils, anaemia and thrombocytopenia. No dysplastic features. No Philadelphia chromosome or BCR-ABL fusion gene. Short history of malaise, sweats, weight loss, skin rash and increased susceptibility to infection. Splenomegaly common. End-organ damage as per HES may occur. Proliferative element may be controlled by hydroxyurea. In symptomatic younger patients consider sibling allogeneic SCT.

• HES is characterised by PB eosinophilia >1.5 x 109/L for >6 months and by end-organ damage, commonly endomyocardial fibrosis, skin lesions (angioedema, urticaria), thromboembolic disease , pulmonary lesions and CNS dysfunction. Associated with polyclonal increased in immunoglobulins including IgE. Splenomegaly 40%. Anaemia 50%; neutrophilia with left shift frequent; platelets may be normal, decreased or increased. BM hypercellular with 25-75% eosinophils with left shift. Eosinophilia may be controlled by prednisolone, hydroxyurea or IFN-a. Allogeneic SCT may be curative in younger patients.

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