U.O. Dermatologia, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milano, Italy.
J Investig Allergol Clin Immunol. 2010;20(7):616-9.
An increased risk of thrombosis has been described in patients with hypereosinophilic syndromes, including Churg-Strauss syndrome (CSS). We report the case of a 43-year-old man with CSS who presented with asthma, pansinusitis, blood eosinophilia (9650/microL), peripheral neuropathy, cutaneous eosinophilic vasculitis, and a positive result for antineutrophil cytoplasmic antibodies. An analysis of plasma during active disease revealed elevated levels of prothrombin fragment 1+2 (marker of thrombin generation) (832 pM; normal range, 68-229 pM) and D-dimer (marker of fibrin degradation) (2300 ng/mL; normal range, 130-250 ng/mL), which indicate an increased risk of thrombosis. Both parameters returned to normal values during remission after immunosuppressive treatment. Skin histology showed leukocytoclastic vasculitis with numerous eosinophils in the dermal infiltrate. Immunohistochemistry revealed expression of tissue factor by skin-infiltrating eosinophils, as confirmed by colocalization with eosinophil cationic protein, a classic marker of eosinophil granulocytes. In conclusion, we present a patient with active CSS and a prothrombotic state that reverted during remission achieved by immunosuppressive therapy.
高嗜酸性粒细胞综合征患者（包括变应性肉芽肿性血管炎）已被描述存在血栓形成风险增加。我们报告了一例 Churg-Strauss 综合征（CSS）患者，该患者表现为哮喘、全鼻窦炎、外周神经病、皮肤嗜酸性血管炎和抗中性粒细胞胞质抗体阳性。在疾病活动期对血浆进行分析显示，凝血酶原片段 1+2（凝血酶生成标志物）（832pM；正常范围 68-229pM）和 D-二聚体（纤维蛋白降解标志物）（2300ng/mL；正常范围 130-250ng/mL）水平升高，表明存在血栓形成风险增加。在免疫抑制治疗缓解后，这两个参数均恢复正常。皮肤组织学显示白细胞碎裂性血管炎，真皮浸润中有大量嗜酸性粒细胞。免疫组化显示组织因子在皮肤浸润的嗜酸性粒细胞中表达，这一点通过与嗜酸性粒细胞阳离子蛋白（嗜酸性粒细胞粒细胞的经典标志物）的共定位得到证实。总之，我们报告了一例患有活动期 CSS 和血栓形成倾向的患者，通过免疫抑制治疗缓解后，该倾向得到逆转。
