T-cell abnormalities are present at high frequencies in patients with hypereosinophilic syndrome

• Published in: Haematologica (Roma) Vol. 94; no. 9; pp. 1236 - 1241
• Main Authors: Helbig, Grzegorz, Wieczorkiewicz, Agata, Dziaczkowska-Suszek, Joanna, Majewski, Miroslaw, Kyrcz-Krzemien, Slawomira
• Format: Journal Article
• Language: English
• Published: Pavia Ferrata Storti Foundation 01-09-2009
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Biological and medical sciences; CD4-CD8 Ratio; Female; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor - genetics; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor - immunology; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor - genetics; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor - immunology; Hematologic and hematopoietic diseases; Humans; Hypereosinophilic Syndrome - blood; Hypereosinophilic Syndrome - genetics; Hypereosinophilic Syndrome - immunology; Male; Medical sciences; Middle Aged; mRNA Cleavage and Polyadenylation Factors - biosynthesis; mRNA Cleavage and Polyadenylation Factors - genetics; mRNA Cleavage and Polyadenylation Factors - immunology; Oncogene Proteins, Fusion - biosynthesis; Oncogene Proteins, Fusion - genetics; Oncogene Proteins, Fusion - immunology; Original; Other diseases. Hematologic involvement in other diseases; Polymerase Chain Reaction; Prospective Studies; Receptor, Platelet-Derived Growth Factor alpha - biosynthesis; Receptor, Platelet-Derived Growth Factor alpha - genetics; Receptor, Platelet-Derived Growth Factor alpha - immunology; T-Lymphocytes - immunology; T-Lymphocytes - metabolism; T-Lymphocytes - pathology; Human; T-cell receptor; Platelet derived growth factor receptor A; T cell receptor; Hematology; T-Lymphocyte; Frequency; Hemopathy; T cells; FIP1L1-PDGFRA; Hypereosinophilic syndrome
• ISSN: 0390-6078; 1592-8721
• DOI: 10.3324/haematol.2008.005447

1 Departament of Haematology and Bone Marrow Transplantation, Silesian Medical University, Katowice
2 Institute of Haematology and Transfusion Medicine, Medical University, Warsaw, Poland
Correspondence: Grzegorz Helbig MD, PhD, Department of Haematology and Bone Marrow Transplantation, Silesian Medical University, Dabrowski Street 25, 40-032 Katowice, Poland., E-mail: ghelbig{at}o2.pl
Background: A T-cell clone, thought to be the source of eosinophilopoietic cytokines, identified by clonal rearrangement of the T-cell receptor and by the presence of aberrant T-cell immunophenotype in peripheral blood defines lymphocytic variant of hypereosinophilic syndrome (L-HES).
Design and Methods: Peripheral blood samples from 42 patients who satisfied the diagnostic criteria for HES were studied for T-cell receptor clonal rearrangement by polymerase chain reaction according to BIOMED-2. The T-cell immunophenotype population was assessed in peripheral blood by flow cytometry. The FIP1L1-PDGFRA fusion gene was detected by nested polymerase chain reaction.
Results: Forty-two HES patients (18 males and 24 females) with a median age at diagnosis of 56 years (range 17–84) were examined in this study. Their median white blood cell count was 12.9 x 10 9 /L (range 5.3–121), with an absolute eosinophil count of 4.5 x 10 9 /L (range 1.5–99) and a median eosinophilic bone marrow infiltration of 30% (range 11–64). Among the 42 patients, clonal T-cell receptor rearrangements were detected in 18 patients (42.8%). Patients with T-cell receptor clonality included: T-cell receptor β in 15 patients (35%), T-cell receptor in 9 (21%) and T-cell receptor in 9 (21%) patients, respectively. Clonality was detected in all three T-cell receptor loci in 4 cases, in two loci in 7 patients and in one T-cell receptor locus in the remaining 7 patients. The FIP1L1-PDGFRA fusion transcript was absent in all but 2 patients with T-cell receptor clonality. Three patients out of 42 revealed an aberrant T-cell immunophenotype. In some patients, an abnormal CD4:CD8 ratio was demonstrated.
Conclusions: T-cell abnormalities are present at high frequencies in patients with HES.
Key words: T cells, T-cell receptor, hypereosinophilic syndrome, FIP1L1-PDGFRA .
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