Galimberti S, Ciabatti E, Ottimo F, Rossi A, Trombi L, Carulli G, Cervetti G, Matti L, Bianchi G, Petrini M
Department of Oncology, Transplant and Advances in Medicine, Section of Hematology, University of Pisa, Ospedale S. Chiara, Pisa Italy.
Clin Exp Rheumatol. 2007 Jan-Feb;25(1):17-22.
Idiopathic hypereosinophilic syndrome (HES) is a heterogeneous disorder, including either a myeloproliferative or a lymphoproliferative variant (l-HES). In l-HES, T-lymphocytes could be involved in the pathogenesis through several cytokines, including IL5.
We assayed both TCR Beta- and delta-rearrangements by fluorescent PCR, characterizing 14 patients affected by HES. Lyn activation (a src-kinase involved in the IL5 pathway) was also tested in 6 cases.
FIP1L1-PDGFRa was detected in 4 cases (28.6%); a clonal TCR was found in 10 cases (71.4%), including cases FIP1L1-PDGFRalpha-positive; four cases did not show any molecular marker. In this series, levels of IL5, IL4, IL2 and gammaIFN were measured, without any significant difference among different subgroups. All pathological samples tested did not show Lyn activation. Immunophenotype was also characterized: only one case showed an atypical CD3-/CD4+ population in the bone marrow.
This study would suggest that a real distinction between m- and l-HES is not wholly convincing and that clonal T-cell expansion could not be the "primum movens" but an epiphenomenon in HES.
特发性嗜酸性粒细胞增多综合征（HES）是一种异质性疾病，包括骨髓增殖性或淋巴细胞增殖性变异型（l-HES）。在l-HES中，T淋巴细胞可能通过包括白细胞介素5（IL5）在内的多种细胞因子参与发病机制。
我们通过荧光聚合酶链反应检测了TCRβ和δ重排，对14例HES患者进行了特征分析。还对6例患者检测了Lyn激活情况（一种参与IL5途径的src激酶）。
4例（28.6%）检测到FIP1L1-PDGFRα；10例（71.4%）发现克隆性TCR，包括FIP1L1-PDGFRα阳性病例；4例未显示任何分子标志物。在该系列中，检测了IL5、IL4、IL2和γ干扰素水平，不同亚组之间无显著差异。所有检测的病理样本均未显示Lyn激活。还对免疫表型进行了特征分析：仅1例骨髓中出现非典型CD3-/CD4+细胞群。
本研究提示，m-HES和l-HES之间的真正区分并不完全令人信服，克隆性T细胞扩增可能不是HES中的“原动者”，而是一种附带现象。
