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泼尼松、甲磺酸伊马替尼和羟基脲联合治疗控制嗜酸性粒细胞增多综合征相关的顽固性冠状动脉痉挛

Control of hypereosinophilic syndrome-associated recalcitrant coronary artery spasm by combined treatment with prednisone, imatinib mesylate and hydroxyurea.

作者信息

Butterfield Joseph H, Sharkey Scott W

机构信息

Division of Allergic Diseases, Mayo Clinic, Rochester.

出版信息

Exp Clin Cardiol. 2006 Spring;11(1):25-8.

PMID:18651015
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2274835/
Abstract

Uncontrolled hypereosinophilic syndrome is frequently associated with cardiovascular consequences that cause significant morbidity and mortality. The present article reports on a patient with hypereosinophilic syndrome in whom recurrent, recalcitrant coronary artery spasm and associated cardiac arrest were the predominant cardiac manifestations. No valvular abnormalities, evidence of mural thrombi or other cardiac findings commonly associated with hypereosinophilic syndrome were detected, and cardiac function remained normal. The serum tryptase level was normal, cysteine-rich hydrophobic domain 2 (CHIC2) deletion analysis of bone marrow cells was negative and no evidence of mastocytosis or other hematological disorder was found in the bone marrow. To allow for the reduction of prednisone, interferon-alpha-2b was added to the patient's program, but caused aggravation of chest pain and was discontinued. However, a combination of reduced prednisone dosage, imatinib mesylate and hydroxyurea successfully controlled the eosinophilia, and thereafter, episodes of coronary artery spasm did not recur. The clinical features of the present case suggest that, in some patients, hypereosinophilia may manifest as resistant coronary artery spasm and that aggressive control of eosinophilia is necessary.

摘要

未控制的嗜酸性粒细胞增多综合征常伴有心血管后果,可导致严重的发病率和死亡率。本文报道了一名嗜酸性粒细胞增多综合征患者,其主要心脏表现为复发性、顽固性冠状动脉痉挛及相关心脏骤停。未检测到瓣膜异常、壁血栓证据或其他通常与嗜酸性粒细胞增多综合征相关的心脏表现,心脏功能保持正常。血清类胰蛋白酶水平正常,骨髓细胞的富含半胱氨酸的疏水结构域2(CHIC2)缺失分析为阴性,骨髓中未发现肥大细胞增多症或其他血液系统疾病的证据。为了减少泼尼松用量,在患者的治疗方案中加入了干扰素-α-2b,但导致胸痛加重,随后停药。然而,泼尼松剂量减少、甲磺酸伊马替尼和羟基脲联合使用成功控制了嗜酸性粒细胞增多,此后冠状动脉痉挛发作未再复发。本病例的临床特征表明,在一些患者中,嗜酸性粒细胞增多可能表现为顽固性冠状动脉痉挛,积极控制嗜酸性粒细胞增多是必要的。

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