Haematologica, Vol 94, Issue 9, 1236-1241 doi:10.3324/haematol.2008.005447
Copyright © 2009 by Ferrata Storti Foundation
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Myeloproliferative Neoplasms

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

Grzegorz Helbig1, Agata Wieczorkiewicz1, Joanna Dziaczkowska-Suszek1, Miroslaw Majewski1, Slawomira Kyrcz-Krzemien1

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 12.9x109/L (range 5.3–121), with an absolute eosinophil count of 4.5x109/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 {gamma} in 9 (21%) and T-cell receptor {delta} 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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Hypereosinophilic syndrome variants: diagnostic and therapeutic considerations
Florence Roufosse
Haematologica 2009 94: 1188-1193. [Full Text] [PDF]



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F. Roufosse
Hypereosinophilic syndrome variants: diagnostic and therapeutic considerations
Haematologica, September 1, 2009; 94(9): 1188 - 1193.
[Full Text] [PDF]