首页> 美国卫生研究院文献>Case Reports in Pancreatic Cancer >The Immune Conundrum: Acquired Hemophilia A Immune Thrombocytopenia and Neutropenia in a Patient with Pancreatic Cancer
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The Immune Conundrum: Acquired Hemophilia A Immune Thrombocytopenia and Neutropenia in a Patient with Pancreatic Cancer

机译:免疫难题:胰腺癌患者获得性血友病A免疫性血小板减少和中性粒细胞减少

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摘要

>Background: Malignancy-associated bleeding can pose diagnostic dilemmas. We report a unique case of paraneoplastic acquired hemophilia A (AHA), immune thrombocytopenia (ITP), and immune neutropenia in a patient with pancreatic adenocarcinoma.>Case Presentation: A 66-year-old male with newly diagnosed pancreatic cancer and normal preoperative hematological evaluation was taken to the operating room for pancreaticoduodenectomy. The operation was aborted due to empyema of the gall bladder, cholangitis, and local extent of disease. Postoperatively, the patient developed bleeding diatheses with mucocutaneous and intra-abdominal bleeding and a prolonged activated partial thromboplastin time. Evaluation revealed high-titer factor VIII inhibitor confirming AHA. Management with bypassing agents such as recombinant activated factor VII, factor VIII inhibitor bypassing activity, and immunosuppression with steroids, cyclophosphamide, and rituximab achieved remission in 2 months. ITP developed after achieving normal factor VIII levels, which was managed with intravenous immunoglobulin. Neutropenia was detected before initiation of chemotherapy and was managed with granulocyte-colony stimulating factor.>Conclusion: These unique challenges posed by paraneoplastic hematological syndromes warrant the need for astute clinical judgment and multidisciplinary collaboration for effective management.
机译:>背景:与恶性肿瘤相关的出血可引起诊断上的两难境地。我们报告了胰腺癌患者副肿瘤性获得性血友病A(AHA),免疫性血小板减少症(ITP)和免疫性中性粒细胞减少的独特病例。>病例介绍:一名66岁男性,新诊断为胰腺癌和术前血液学评估正常后被送至手术室进行胰十二指肠切除术。手术因胆囊积脓,胆管炎和局部疾病而中止。术后,患者出现了因皮肤粘膜和腹腔内出血而出血的情况,并导致部分凝血活酶时间延长。评估显示,高滴定因子VIII抑制剂证实了AHA。使用绕过剂(例如重组活化的VII因子,VIII因子抑制剂的绕过活性)以及类固醇,环磷酰胺和利妥昔单抗的免疫抑制可在2个月内缓解。在达到正常的VIII因子水平后,ITP发生了发展,可以通过静脉注射免疫球蛋白进行控制。嗜中性白血球减少症在开始化疗之前就已检测到,并通过粒细胞集落刺激因子进行治疗。>结论:副肿瘤性血液综合症所带来的这些独特挑战,需要对临床进行明智的判断和多学科协作以进行有效管理。

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