Disease relevance of Hypereosinophilic Syndrome

• Entities encountered included: 1) constrictive pericarditis; 2) Loeffler's endocarditis; 3) Ebstein's anomaly with tricuspid regurgitation; 4) tricuspid regurgitation following tricuspid valvulectomy, and 5) pulmonary regurgitation accompanied by atrial septal defect [1].
• Since serum tryptase levels are elevated in some patients with myeloproliferative disorders, we examined their utility in identifying a subset of patients with hypereosinophilic syndrome (HES) and an underlying myeloproliferative disorder [2].
• Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness [2].
• Bone marrow-derived genomic DNA from 245 patients--119 with chronic myelomonocytic leukemia (CMML), 101 with MDS, 11 with hypereosinophilic syndrome (HES), 8 with systemic mastocytosis (SM), and 6 with chronic neutrophilic leukemia (CNL)--was screened for the JAK2 V617F mutation [3].
• Autocrine PDGF receptor signaling occurs in certain malignancies characterized by mutational activation of PDGF or PDGF receptors, for instance, dermatofibrosaracoma protuberans, gastrointestinal stromal tumors, and hypereosinophilic syndrome [4].

High impact information on Hypereosinophilic Syndrome

• Troleandomycin and methylprednisolone for treatment of the hypereosinophilic syndrome [5].
• Acute hypereosinophilic syndrome. Successful treatment with vincristine, cytarabine, and prednisone [6].
• Demonstration of TGF-alpha production by tissue-infiltrating eosinophils and the eosinophils in the hypereosinophilic syndrome identifies a novel mechanism by which eosinophils might contribute to physiological, immunological, and pathological responses [7].
• The soluble MBP immunoreactivities in placental homogenates and in maternal serum chromatograph identically on Sephadex G-50, and both these gestational MBP molecules migrate as though substantially larger than the MBP found in serum from patients with hypereosinophilic syndrome or purified from the eosinophil granule [8].
• These data demonstrate that induction of lymphoid chimerism in the absence of MHC class I-T-cell interactions results in a lethal form of host-versus-graft disease that represents a unique model of Th2-dependent chronic inflammatory disease associated with an hypereosinophilic syndrome in mice [9].

Chemical compound and disease context of Hypereosinophilic Syndrome

• Treatment of hypereosinophilic syndrome with imatinib mesilate [10].
• Imatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders [11].
• The FIP1L1-PDGFRalpha fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management [12].
• Effect of docosahexaenoic acid on the generation of platelet-activating factor by eosinophilic leukemia cells, Eol-1 [13].
• Eosinophilic leukemia. Remission with vincristine and hydroxyurea [14].

Biological context of Hypereosinophilic Syndrome

• The FIP1L1-PDGFRA fusion gene cooperates with IL-5 to induce murine hypereosinophilic syndrome (HES)/chronic eosinophilic leukemia (CEL)-like disease [15].
• In four cases of eosinophilic diseases with severe cardiac failure, we evaluated the proportion of eosinophil phenotypes and the serum levels of eosinophil cationic protein (ECP) and soluble IL-2 receptor (sIL-2R), markers of disease activity in the hypereosinophilic syndromes [16].
• Human chymase induced a rapid phosphorylation of ERK1/2 and p38 in human eosinophilic leukemia EoL-1 cells, while no phosphorylation was detected in JNK [17].
• Cytogenetic remission with imatinib therapy in hypereosinophilic syndrome with trisomy 8 and resolution of severe cardiac dysfunction [18].

Anatomical context of Hypereosinophilic Syndrome

• Two types of activation Ag, low affinity FcR for IgE (Fc epsilon R2)/CD23 and IL-2R (Tac/p55), were expressed and differently regulated on human eosinophilic leukemia cell lines (EoL-1 and EoL-3) [19].
• Elevated production of interleukin-4 and interleukin-5 by T cells in a child with idiopathic hypereosinophilic syndrome [20].
• High serum thymus and activation-regulated chemokine levels in the lymphocytic variant of the hypereosinophilic syndrome [21].
• Eosinophil degranulation induced by the calcium ionophore A23187 and opsonized zymosan particles was examined ultrastructurally in peripheral blood cells obtained from patients with the hypereosinophilic syndrome [22].
• A 33-year-old man with an atypical course of hypereosinophilic syndrome including malignant hypercalcemia, osteolytic lesions and evolution into severe myelofibrosis was treated by allogeneic bone marrow transplantation after conditioning with cytoxan and total body irradiation [23].

Gene context of Hypereosinophilic Syndrome

• We recently identified the chimeric kinase FIP1L1-platelet-derived growth factor receptor alpha (PDGFRalpha) as a cause of the hypereosinophilic syndrome and of chronic eosinophilic leukemia [24].
• A human cDNA encoding a putative G protein-coupled receptor designated chemokine beta receptor-like 1 (CMKBRL1) was isolated from an eosinophilic leukemia library [25].
• Induction of eosinophilic granules, nonspecific esterase activity and CD14 expression in the human eosinophilic leukemia cell line, EOL-1 [26].
• A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome [27].
• We report a case of hypereosinophilic syndrome in which the presence of a population of CD3-CD4+ cells able to overproduce IL-5 was shown [28].

Analytical, diagnostic and therapeutic context of Hypereosinophilic Syndrome

• By Northern blot analysis, highly enriched blood eosinophils from a patient with the idiopathic hypereosinophilic syndrome exhibited differential expression of TNF-alpha and MIP-1 alpha mRNA [29].
• Expression of iNOS protein was also detected by immunoblotting in human purified eosinophils and an eosinophilic leukemia cell line, Eol-3 [30].
• Sustained molecular response with imatinib in a leukemic form of idiopathic hypereosinophilic syndrome in relapse after allograft [31].
• This is the first case of hypereosinophilic syndrome induced by nafamostat mesilate in a hemodialysis patient [32].
• Examples include the use of STI571 in patients with SM plus hypereosinophilic syndrome (SM-HES) and the FIPL1/PDGFRA fusion gene target, or chemotherapy for eradication of AML in patients with SM-AML [33].

References