Hussain Shabneez, Adil Salman Naseem, Sami Shahid Ahmed
Section of Haematology, Department of Pathology and Microbiology, The Aga Khan University Hospital, Stadium Road, P.O. Box 3500, Karachi, 74800, Pakistan.
Fatimid Foundation, 393, Britto Road, Garden east, Karachi, 74800, Pakistan.
BMC Res Notes. 2015 Oct 22;8:594. doi: 10.1186/s13104-015-1572-3.
Idiopathic aortitis is among the most common causes of non-infectious aortitis, which rarely presents with anemia.
Here we report a case of a 49-year-old muhajir female who presented with shortness of breath and easy fatigability for the past 6 months. Physical examination revealed pallor and a diastolic murmur in the aortic region. Echocardiography showed thickened and calcified aortic and mitral valves, severe aortic regurgitation and dilatation of ascending aorta. She was advised aortic valve replacement and was referred to a haematologist due to concomitant anemia. Complete blood counts revealed haemoglobin: 7.7 gm/dl, mean corpuscular volume (MCV): 78 fl, mean corpuscular haemoglobin (MCH):23 pg, total white cell count: 9.0 × 10(9)/L and platelet count: 227 × 10(9)/L. Erythrocyte sedimentation rate (ESR) was 100 mm/hr. There was suspicion of myelodysplastic syndrome, but could not be confirmed as the patient refused bone marrow and cytogenetic studies. She was given erythropoietin, folic acid and ferrous sulphate. Following relatively prolonged therapy, her haemoglobin level increased to approximately 9.0 gm/dL. She was transfused with packed red cells and underwent aortic valve and ascending aorta replacement. The ascending aorta was dilated and aortic wall markedly thick and irregular. Histopathology of the resected aorta revealed granulomatous aortitis. She was prescribed prednisolone, which resulted in further incremental rise of haemoglobin to 13.1 gm/dL. One month later, she developed complaints of blurred vision in the right eye and was diagnosed with central retinal vein occlusion. She was treated with antiplatelet agents and her vision improved. After 3 months, she was asymptomatic and her haemoglobin level rose to 11.2 gm/dL without hematinic therapy or blood transfusion. She was begun on anticoagulant therapy and remains clinically stable.
We report a case of idiopathic aortitis with presumed diagnosis of anemia of chronic disease exhibiting a transient response towards steroid therapy post-valvuloplasty.
特发性主动脉炎是非感染性主动脉炎最常见的病因之一,很少表现为贫血。
我们在此报告一例49岁的穆哈吉女性患者,她在过去6个月出现呼吸急促和易疲劳症状。体格检查发现面色苍白,主动脉区有舒张期杂音。超声心动图显示主动脉瓣和二尖瓣增厚、钙化,严重主动脉瓣反流及升主动脉扩张。建议她进行主动脉瓣置换术,因其合并贫血转诊至血液科医生处。全血细胞计数显示血红蛋白:7.7克/分升,平均红细胞体积(MCV):78飞升,平均红细胞血红蛋白(MCH):23皮克,白细胞总数:9.0×10⁹/L,血小板计数:227×10⁹/L。红细胞沉降率(ESR)为100毫米/小时。怀疑为骨髓增生异常综合征,但因患者拒绝骨髓及细胞遗传学检查而无法确诊。给予她促红细胞生成素、叶酸和硫酸亚铁。经过相对较长时间的治疗,她的血红蛋白水平升至约9.0克/分升。输注浓缩红细胞后,她接受了主动脉瓣和升主动脉置换术。升主动脉扩张,主动脉壁明显增厚且不规则。切除的主动脉组织病理学检查显示为肉芽肿性主动脉炎。给予泼尼松龙治疗,血红蛋白进一步升至13.1克/分升。1个月后,她出现右眼视物模糊的症状,被诊断为视网膜中央静脉阻塞。给予抗血小板药物治疗后视力改善。3个月后,她无症状,血红蛋白水平升至11.2克/分升,无需补血治疗或输血。开始给予抗凝治疗,她目前临床稳定。
我们报告一例特发性主动脉炎病例,推测诊断为慢性病贫血,瓣膜成形术后对类固醇治疗呈现短暂反应。