Hypereosinophilic syndrome and clonal eosinophilia: point-of-care diagnostic algorithm and treatment update.
Acquired eosinophilia is operationally categorized into secondary, clonal, and idiopathic types. Causes of secondary eosinophilia include parasite infections, allergic or vasculitis conditions, drugs, and lymphoma. Clonal eosinophilia is distinguished from idiopathic eosinophilia by the presence of histologic, cytogenetic, or molecular evidence of an underlying myeloid malignancy. The World Health Organization classification system for hematologic malignancies recognizes 2 distinct subcategories of clonal eosinophilia: chronic eosinophilic leukemia, not otherwise specified and myeloid/lymphoid neoplasms with eosinophilia and mutations involving platelet-derived growth factor receptor alpha/beta or fibroblast growth factor receptor 1. Clonal eosinophilia might also accompany other World Health Organization-defined myeloid malignancies, including chronic myelogenous leukemia, myelodysplastic syndromes, chronic myelomonocytic leukemia, and systemic mastocytosis. Hypereosinophilic syndrome, a subcategory of idiopathic eosinophilia, is defined by the presence of a peripheral blood eosinophil count of 1.5 x 10(9)/L or greater for at least 6 months (a shorter duration is acceptable in the presence of symptoms that require eosinophil-lowering therapy), exclusion of both secondary and clonal eosinophilia, evidence of organ involvement, and absence of phenotypically abnormal and/or clonal T lymphocytes. The presence of the latter defines lymphocytic variant hyper eosinophilia, which is best classified under secondary eosinophilia. In the current review, we provide a simplified algorithm for distinguishing the various causes of clonal and idiopathic eosinophilia and discuss current therapy, including new drugs (imatinib mesylate, alemtuzumab, and mepolizumab).
Tefferi
Ayalew
A
Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA. tefferi.ayalew@mayo.edu
Gotlib
Jason
J
Pardanani
Animesh
A
D000074323:Alemtuzumab; D000465:Algorithms; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides; D015984:Causality; D003663:Decision Trees; D003937:Diagnosis, Differential; D004802:Eosinophilia / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D000068877:Imatinib Mesylate; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000150:complications; D015477:Leukemia, Myelomonocytic, Chronic / Q000150:complications; D008415:Mastocytosis / Q000150:complications; D009154:Mutation / Q000235:genetics; D009190:Myelodysplastic Syndromes / Q000150:complications; D010879:Piperazines / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D051496:Receptor, Fibroblast Growth Factor, Type 1 / Q000235:genetics; D017479:Receptors, Platelet-Derived Growth Factor / Q000235:genetics; D039221:mRNA Cleavage and Polyadenylation Factors / Q000235:genetics
D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm; D000970:Antineoplastic Agents; D001549:Benzamides; C479818:FIP1L1 protein, human; D010879:Piperazines; D011743:Pyrimidines; D039221:mRNA Cleavage and Polyadenylation Factors; D000074323:Alemtuzumab; D000068877:Imatinib Mesylate; C434107:mepolizumab; D051496:Receptor, Fibroblast Growth Factor, Type 1; D017479:Receptors, Platelet-Derived Growth Factor