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布加综合征合并自身免疫性溶血性贫血、免疫性血小板减少性紫癜和狼疮性肾炎:病例报告

Budd-Chiari Syndrome Associated With Autoimmune Hemolytic Anemia, Immune Thrombocytopenic Purpura, and Lupus Nephritis: A Case Report.

作者信息

Sah Soni K, Maurya Archana, Weerarathna Induni N

机构信息

Nursing, Srimati Radhikabai Meghe Memorial College of Nursing, Datta Meghe Institute of Higher Education and Research, Wardha, IND.

Child Health Nursing, Srimati Radhikabai Meghe Memorial College of Nursing, Datta Meghe Institute of Higher Education and Research, Wardha, IND.

出版信息

Cureus. 2024 Jul 22;16(7):e65160. doi: 10.7759/cureus.65160. eCollection 2024 Jul.

Abstract

Budd-Chiari syndrome (BCS) is a scarce but severe condition characterized by the obstruction of the hepatic veins, liver congestion, and consequent damage. This series brings up one unusual presentation of BCS associated with autoimmune hemolytic anemia (AIHA), immune thrombocytopenic purpura (ITP), and lupus nephritis (LN), which collectively complicate the clinical scenario. This is a 19-year-old woman who was admitted for abdominal pain, hepatomegaly, ascites, and jaundice. Her history included the diagnosis of systemic lupus erythematosus. Laboratory findings revealed hemolytic anemia, thrombocytopenia, and impaired renal function. Imaging investigations were done to prove the diagnosis of BCS. The patient's complex autoimmune profile, characterized by the simultaneous presence of AIHA and ITP with LN, underlined the multifaceted nature of her condition. This case underscores the diagnostic and therapeutic challenges posed by the co-existence of BCS with AIHA, ITP, and LN, highlighting the critical role of a multidisciplinary approach in managing such complex cases effectively. Timely diagnosis and targeted treatment strategies are essential for improving outcomes in these patients.

摘要

布加综合征(BCS)是一种罕见但严重的疾病,其特征为肝静脉阻塞、肝脏充血及由此导致的损害。本系列报道了1例不寻常的布加综合征,伴有自身免疫性溶血性贫血(AIHA)、免疫性血小板减少性紫癜(ITP)和狼疮性肾炎(LN),这些情况共同使临床病情复杂化。这是一名19岁女性,因腹痛、肝肿大、腹水和黄疸入院。她有系统性红斑狼疮的诊断史。实验室检查发现溶血性贫血、血小板减少和肾功能受损。进行了影像学检查以证实布加综合征的诊断。患者复杂的自身免疫状况,表现为同时存在AIHA、ITP和LN,突显了其病情的多面性。该病例强调了布加综合征与AIHA、ITP和LN并存所带来的诊断和治疗挑战,突出了多学科方法在有效管理此类复杂病例中的关键作用。及时诊断和针对性治疗策略对于改善这些患者的预后至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f6c7/11341066/c11311ffca7e/cureus-0016-00000065160-i01.jpg

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