Helbig Grzegorz, Wieczorkiewicz Agata, Dziaczkowska-Suszek Joanna, Majewski Miroslaw, Kyrcz-Krzemien Slawomira
Department of Haematology and Bone Marrow Transplantation, Silesian Medical University, Dabrowski Street 25, Katowice, Poland.
Haematologica. 2009 Sep;94(9):1236-41. doi: 10.3324/haematol.2008.005447.
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).
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.
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 beta 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.
T-cell abnormalities are present at high frequencies in patients with HES.
一个T细胞克隆，被认为是嗜酸性粒细胞生成细胞因子的来源，通过T细胞受体的克隆重排以及外周血中异常T细胞免疫表型的存在来确定，这定义了高嗜酸性粒细胞综合征的淋巴细胞变异型（L-HES）。
对42例符合HES诊断标准的患者的外周血样本，根据BIOMED-2通过聚合酶链反应研究T细胞受体克隆重排。通过流式细胞术对外周血中的T细胞免疫表型群体进行评估。通过巢式聚合酶链反应检测FIP1L1-PDGFRA融合基因。
本研究检查了42例HES患者（18例男性和24例女性），诊断时的中位年龄为56岁（范围17 - 84岁）。他们的中位白细胞计数为12.9×10⁹/L（范围5.3 - 121），绝对嗜酸性粒细胞计数为4.5×10⁹/L（范围1.5 - 99），骨髓嗜酸性粒细胞浸润的中位值为30%（范围11 - 64）。在42例患者中，18例（42.8%）检测到克隆性T细胞受体重排。具有T细胞受体克隆性的患者包括：15例（35%）患者的T细胞受体β、9例（21%）患者的T细胞受体γ和9例（21%）患者的T细胞受体δ。4例患者在所有三个T细胞受体基因座检测到克隆性，7例患者在两个基因座检测到克隆性，其余7例患者在一个T细胞受体基因座检测到克隆性。除2例具有T细胞受体克隆性的患者外，所有患者均未检测到FIP1L1-PDGFRA融合转录本。42例患者中有3例显示异常T细胞免疫表型。在一些患者中，出现了异常的CD4:CD8比值。
HES患者中T细胞异常的发生率很高。
