L-HES is a rare, clonal lymphoproliferation of abnormal memory T cells that produces similar clinical symptoms as SS, including severe pruritus and eosinophilia.
The revised 2008 WHO classification system featured the following changes: 1) the term "CMPD" was replaced by "myeloproliferative neoplasm (MPN)," 2) mast cell disease was formally included under the category of MPN, and 3) the subcategory of CEL/HES was reorganized into "CEL not otherwise specified (CEL-NOS)" and "myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, and FGFR1"; CEL-NOS remained a subcategory of "MPN," whereas the latter neoplasms were now assigned a new category of their own.
Recent studies examining eosinophil biology have focused on delineating the molecular basis of FIP1L1/PDGRFalpha-fusion gene induced HES, the molecular steps involved in eosinophil recruitment in tumor-associated eosinophilia and EGID, and the role of eosinophils in asthma.
Hypereosinophilic syndromes may result either from eosinophilic differentiation of a clone of neoplastic cells or from reactive eosinophilia.In other patients HES is idiopathic.