伊马替尼治疗PDGFRA重排引起的克隆性嗜酸性粒细胞增多伴排他性肺部受累1例

IF 0.7 Q4 HEMATOLOGY
Zaineb Mlayah, Inés Ben-Rekaya, Inaam Bizid, Nader Slama, Sara Boukhris, Mohamed-Adnene Laatiri
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引用次数: 0

摘要

高嗜酸性粒细胞综合征(HES)在1968年由Hardy和Anderson首次描述。它是一组罕见的、多系统的、异质性的病理,以显著的发病率和死亡率为特征。与FIP 1L1-PDGFRA+相关的克隆性高嗜酸性粒细胞综合征的发生率估计在每百万人0.31至6.3例之间。最常受影响的器官是心脏和脾脏,其次是肺部。克隆性高嗜酸性粒细胞综合征只累及肺部是例外。由于克隆性HES罕见,本报告旨在描述克隆性HES患者的临床、生物学和放射学表现,同时丰富文献以改善这种罕见综合征的治疗。我们报告的情况下,病人过去的病史阻塞性支气管肺炎谁是咳嗽和呼吸困难的表现。调查显示外周血嗜酸性粒细胞增多(4000 - 9000/mm3),导致我们怀疑克隆性嗜酸性粒细胞增多综合征(HES)。细胞遗传学/荧光原位杂交(FISH)证实了这一诊断,显示FIP 1L1-PDGFRA重排阳性。CTAP证实孤立性肺受累,双肺基底胸膜下区域间质浸润,支气管肺泡灌洗显示嗜酸性粒细胞计数升高15%。患者开始接受伊马替尼治疗,剂量为100mg /天。患者随访显示,治疗两个月后,嗜酸性粒细胞计数降至7500/mm3。计划在3个月内进行分子评估以评估对伊马替尼的反应。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clonal eosinophilia with exclusive pulmonary involvement driven by PDGFRA rearrangement treated with imatinib: A case report
Hypereosinophilic syndrome (HES) was first described in 1968 by Hardy and Anderson. It is a group of rare, multisystemic and heterogeneous pathologies, characterized by significant morbidity and mortality. The occurrence of clonal hypereosinophilic syndrome associated with FIP 1L1-PDGFRA+ is estimated to range between 0.31 and 6.3 cases per million individuals. The organs most commonly impacted are the heart and spleen, with the lungs being the next most affected. Clonal hypereosinophilic syndromes with exclusive pulmonary involvement are exceptional. Due to the rarity of clonal HES, this report aims to not only describe the patient's clinical, biological, and radiological manifestations of clonal HES but also enrich the literature to ameliorate the management of this uncommon syndrome.
we report the case of a patient with past medical history of obstructive bronchopneumopathy who was presented with cough and dyspnea. Investigations revealed peripheral blood hypereosinophilia (between 4000 and 9000/mm3) which lead us to suspect clonal hypereosinophilic syndrome (HES). This diagnosis was confirmed by cytogenetics/fluorescence in situ hybridization (FISH) which demonstrated a positive FIP 1L1-PDGFRA rearrangement. The CTAP confirmed isolated lung involvement with interstitial infiltrate of the subpleural territories of both lung bases and the bronchoalveolar lavage showed eosinophil count elevated at 15%. The patient was treated by imatinib at a dose of 100 mg/day was initiated. The patient follow-up showed a reduction in eosinophils count to 7500/mm3 at two months of treatment. A molecular evaluation is scheduled in 3 months to assess the response to imatinib.
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来源期刊
Leukemia Research Reports
Leukemia Research Reports Medicine-Oncology
CiteScore
1.70
自引率
0.00%
发文量
70
审稿时长
23 weeks
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