Uddin Syed Mohammad Mazhar, Haq Aatera, Haq Zara, Yaqoob Uzair
Civil Hospital, Karachi, Sindh, Pakistan.
Dow University of Health Sciences, Karachi, Pakistan.
F1000Res. 2019 Feb 14;8:181. doi: 10.12688/f1000research.18182.1. eCollection 2019.
Celiac disease is an immune-mediated enteropathy due to permanent sensitivity to gluten in genetically predisposed individuals. Evans syndrome is an autoimmune disorder designated with simultaneous or successive development of autoimmune hemolytic anemia and immune thrombocytopenia and/or immune neutropenia in the absence of any cause. We report a rare case of Celiac disease and Evans syndrome in a 20-year-old female who presented to us with generalized weakness and shortness of breath. Her examination finding included anemia, jaundice, and raised jugular venous pulse. Her abdominal exam revealed hepatosplenomegaly. Her laboratory values showed microcytic anemia, leukocytosis and thrombocytopenia. To rule out secondary causes of idiopathic thrombocytopenia purpura, we tested viral markers for Human immunodeficiency virus, Epstein bar virus, Cytomegalovirus and performed a test, all of which were negative. We also ruled out idiopathic thrombocytopenia purpura associated with any thyroid disorder. For celiac disease, we took anti-tissue transgulataminase titers of IgA and IgG which confirmed the diagnosis of celiac disease. For the diagnosis of Evans syndrome, despite a negative serum coombs test initially, her bone marrow sample showed a positive Coombs test along with immune mediated hemolytic anemia and immune mediated thrombocytopenia. The patient was treated with prednisone which was tapered off and counseling was provided regarding a gluten free diet. Although rare, tests for Evans syndrome (and other coexisting autoimmune problems) should be performed in patients with celiac disease.
乳糜泻是一种免疫介导的肠病，发生于对麸质具有永久敏感性的遗传易感性个体。伊文氏综合征是一种自身免疫性疾病，其特征为在无任何病因的情况下，同时或相继发生自身免疫性溶血性贫血和免疫性血小板减少症和/或免疫性中性粒细胞减少症。我们报告了一例罕见的乳糜泻合并伊文氏综合征病例，患者为一名20岁女性，因全身乏力和气促前来就诊。她的检查结果包括贫血、黄疸和颈静脉压升高。腹部检查发现肝脾肿大。实验室检查结果显示小细胞贫血、白细胞增多和血小板减少。为排除特发性血小板减少性紫癜的继发原因，我们检测了人类免疫缺陷病毒、EB病毒、巨细胞病毒的病毒标志物并进行了一项检测，所有结果均为阴性。我们还排除了与任何甲状腺疾病相关的特发性血小板减少性紫癜。对于乳糜泻，我们检测了抗组织转谷氨酰胺酶IgA和IgG抗体滴度，确诊为乳糜泻。对于伊文氏综合征的诊断，尽管最初血清库姆斯试验为阴性，但她的骨髓样本显示库姆斯试验阳性，同时伴有免疫介导的溶血性贫血和免疫介导的血小板减少症。患者接受了泼尼松治疗，随后逐渐减量，并就无麸质饮食提供了咨询。尽管罕见，但对于乳糜泻患者，应进行伊文氏综合征（以及其他并存的自身免疫问题）的检测。
