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Diagnostic and therapeutic management of eosinophilia-associated chronic myeloproliferative disorders

Diagnostic and therapeutic management of eosinophilia-associated chronic myeloproliferative disorders
Diagnostic and therapeutic management of eosinophilia-associated chronic myeloproliferative disorders
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.
humans, research support, myeloproliferative disorders, diagnosis, editorial, eosinophilia, complications, therapy, research
0390-6078
1153-1158
Reiter, Andreas
ffa23e84-4a13-4cb5-aaf0-3fafe25dbede
Grimwade, David
9f299c71-1e4c-42b1-9b39-d1e1ddc1dc26
Cross, Nicholas C.P.
f87650da-b908-4a34-b31b-d62c5f186fe4
Reiter, Andreas
ffa23e84-4a13-4cb5-aaf0-3fafe25dbede
Grimwade, David
9f299c71-1e4c-42b1-9b39-d1e1ddc1dc26
Cross, Nicholas C.P.
f87650da-b908-4a34-b31b-d62c5f186fe4

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).

Record type: Article

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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More information

Published date: July 2007
Keywords: humans, research support, myeloproliferative disorders, diagnosis, editorial, eosinophilia, complications, therapy, research

Identifiers

Local EPrints ID: 60158
URI: http://eprints.soton.ac.uk/id/eprint/60158
ISSN: 0390-6078
PURE UUID: f8092f6f-d0e5-4429-ac41-8bb88d2707ef
ORCID for Nicholas C.P. Cross: ORCID iD orcid.org/0000-0001-5481-2555

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Date deposited: 05 Sep 2008
Last modified: 16 Mar 2024 03:23

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Author: Andreas Reiter
Author: David Grimwade

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