Mansour Maryam, Shamasnah Arwa, Alsaadi Deema, Abu Saif Saja, Krama Akram
Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.
Istishari Arab Hospital, Ramallah, Palestine.
J Med Case Rep. 2024 Dec 28;18(1):643. doi: 10.1186/s13256-024-05002-3.
Evans syndrome is a rare disorder characterized by the simultaneous or sequential combination of autoimmune hemolytic anemia and immunological thrombocytopenia, together with a positive direct antiglobulin test. This syndrome, which can be primary or secondary, is a rare initial manifestation of autoimmune diseases, notably systemic lupus erythematosus, with 1.7-2.7% of patients with systemic lupus erythematosus developing secondary Evans syndrome, sometimes preceding the onset of systemic lupus erythematosus symptoms.
A 47-year-old Middle Eastern female presented with symptoms including shortness of breath, chest pain, and weakness. Physical examination revealed pallor, pale conjunctiva, icteric sclera, tachycardia, and tachypnea. She was diagnosed with Evans syndrome owing to hemolytic anemia, thrombocytopenia, and a positive Coombs test, where initial resistance to treatment prompted intensive therapy with methylprednisolone, intravenous immunoglobulin, and rituximab. Subsequent identification of systemic lupus erythematosus on the basis of positive anti-nuclear antibodies and anti-double-stranded deoxyribonucleic acid antibodies led to treatment with mycophenolate mofetil and hydroxychloroquine. Further evaluations uncovered Hashimoto's thyroiditis and pernicious anemia, necessitating thyroxine supplementation and vitamin B12 injections.
Hematologic abnormalities play a crucial role in the diagnostic framework of systemic lupus erythematosus. This case highlights a patient initially diagnosed with Evans syndrome, revealing an underlying systemic lupus erythematosus. The presentation underscores the significance of hematologic manifestations as integral components of the diagnostic framework for autoimmune diseases, emphasizing the intricate relationship between Evans syndrome and systemic lupus erythematosus.
伊文氏综合征是一种罕见的疾病，其特征为自身免疫性溶血性贫血和免疫性血小板减少症同时或相继出现，且直接抗球蛋白试验呈阳性。该综合征可分为原发性或继发性，是自身免疫性疾病罕见的初始表现，尤其是系统性红斑狼疮，系统性红斑狼疮患者中有1.7%-2.7%会发展为继发性伊文氏综合征，有时在系统性红斑狼疮症状出现之前。
一名47岁的中东女性出现了呼吸急促、胸痛和虚弱等症状。体格检查发现面色苍白、结膜苍白、巩膜黄染、心动过速和呼吸急促。由于溶血性贫血、血小板减少症以及库姆斯试验呈阳性，她被诊断为伊文氏综合征，最初对治疗的抵抗促使采用甲泼尼龙、静脉注射免疫球蛋白和利妥昔单抗进行强化治疗。随后基于抗核抗体和抗双链脱氧核糖核酸抗体呈阳性确诊为系统性红斑狼疮，从而采用霉酚酸酯和羟氯喹进行治疗。进一步评估发现了桥本甲状腺炎和恶性贫血，需要补充甲状腺素和注射维生素B12。
血液学异常在系统性红斑狼疮的诊断框架中起着关键作用。本病例突出了一名最初被诊断为伊文氏综合征的患者，揭示了潜在的系统性红斑狼疮。该病例强调了血液学表现作为自身免疫性疾病诊断框架组成部分的重要性，强调了伊文氏综合征与系统性红斑狼疮之间的复杂关系。
