Diagnostic and therapeutic management of eosinophilia-associated chronic myeloproliferative disorders

Reiter, Andreas, Grimwade, David and Cross, Nicholas C.P. (2007) Diagnostic and therapeutic management of eosinophilia-associated chronic myeloproliferative disorders. Haematologica, 92 (9), 1153-1158. (doi:10.3324/haematol.10328).

Abstract

Eosinophilia is commonly observed in a wide range of disparate non-clonal and clonal disorders.1,2 In the majority of cases it is reactive, associated with atopic conditions, autoimmune disorders, infections or malignancies. In rare cases, a hematologic disorder underlies sustained eosinophilia which can be either non-clonal or clonal. The former (secondary eosinophilia) can be found in a variety of hematologic malignancies including Hodgkin’s disease and lymphomas, predominantly of T-cell phenotype. Hypereosinophilic syndrome (HES) is diagnosed when the blood eosinophil count is persistently greater than 1500/µL for at least 6 months with no evidence of a reactive condition or clonality. HES is a potentially life-threatening condition associated with end-organ damage to heart, gastrointestinal tract, skin, joints or nervous system due to release of granular contents from infiltrating eosinophils. In the lymphocytic variant (L-HES), clonal T-lymphocytes induce non-clonal eosinophil proliferation through overproduction of eosinophilopoietic cytokines such as IL-3, IL-5 or GM-CSF. Clonal or primary eosinophilia is generally associated with chronic myeloproliferative disorders (Eos-MPD), including atypical chronic myeloid leukemia (aCML), myeloproliferative variant of HES (M-HES), chronic myelomonocytic leukemia (CMML), unclassifiable overlap syndromes of myelodysplastic syndrome/myeloproliferative disorders (MDS/MPD) and systemic mastocytosis (SM). Chronic eosinophilic leukemia (CEL) is diagnosed in the presence of increased numbers of blasts and/or proof of clonality through cytogenetic or molecular analyses.

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Contributors

Author: Nicholas C.P. Cross

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