Law Arjun Datt, Varma Subhash, Varma Neelam, Khadwal Alka, Prakash Gaurav, Suri Vikas, Kumari Savita, Malhotra Pankaj
Department of Internal Medicine, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160 012 India.
Department of Haematology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160 012 India.
Indian J Hematol Blood Transfus. 2014 Sep;30(Suppl 1):325-30. doi: 10.1007/s12288-014-0384-2. Epub 2014 Apr 16.
Hypereosinophilia is part of a group of complex disorders with multisystem involvement. A 23 year old male was admitted to our centre with bilateral popliteal artery and venous thrombosis and impending gangrene of the left forefoot along with deep venous thrombosis of the right lower extremity. Investigations revealed marked peripheral blood eosinophilia (27,669/μL). Bone marrow showed increased eosinophils & eosinophil precursors and no evidence of a clonal disorder. Skin biopsy from the ulcerated lesions showed small vessel vasculitis with intense eosinophilic infiltration. Investigations to look for secondary causes of hypereosinophilia in the form of Antinuclear Antibody, P-Anti Neutrophil Cytoplasmic Antibody (ANCA) and C-ANCA and FIP1L1-PDGFRA, Bcr-Abl and JAK2V617F mutations were negative. The arterial and venous thrombosis and cutaneous vasculitis were linked to the presence of hypereosinophilic syndrome. The patient's illness responded to high dose corticosteroids leading to complete resolution of symptoms. We reviewed the literature on the lesser known entity of eosinophilic vasculitis and its association with thrombosis.
嗜酸性粒细胞增多症是一组累及多系统的复杂疾病的一部分。一名23岁男性因双侧腘动脉和静脉血栓形成、左前足即将发生坏疽以及右下肢深静脉血栓形成而入住我们中心。检查发现外周血嗜酸性粒细胞显著增多（27,669/μL）。骨髓显示嗜酸性粒细胞和嗜酸性粒细胞前体增多，且无克隆性疾病证据。溃疡病变的皮肤活检显示小血管血管炎伴强烈嗜酸性粒细胞浸润。以抗核抗体、P-抗中性粒细胞胞浆抗体（ANCA）和C-ANCA以及FIP1L1-PDGFRA、Bcr-Abl和JAK2V617F突变形式寻找嗜酸性粒细胞增多症继发原因的检查均为阴性。动脉和静脉血栓形成以及皮肤血管炎与嗜酸性粒细胞增多综合征的存在有关。患者的病情对大剂量皮质类固醇有反应，症状完全缓解。我们回顾了关于嗜酸性粒细胞性血管炎这一鲜为人知的实体及其与血栓形成关联的文献。
