“免疫难题”:获得性血友病A、免疫性血小板减少症和中性粒细胞减少症在胰腺癌患者。

Praveen Ramakrishnan Geethakumari, Ashwin Sama, Jaime G Caro, Charles J Yeo, Srikanth Nagalla
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引用次数: 3

摘要

背景:恶性肿瘤相关出血可造成诊断困境。我们报告一个独特的病例副肿瘤性获得性血友病a (AHA),免疫性血小板减少症(ITP),和免疫中性粒细胞减少症的胰腺腺癌患者。病例介绍:66岁男性,新诊断为胰腺癌,术前血液学检查正常,被送往手术室行胰十二指肠切除术。手术因胆囊脓肿、胆管炎及局部病变而流产。术后,患者出现出血性糖尿病伴皮肤粘膜和腹腔内出血,激活部分凝血活酶时间延长。评估显示高滴度因子VIII抑制剂证实AHA。使用重组活化因子7、因子8抑制剂等旁路药物,以及类固醇、环磷酰胺和利妥昔单抗的免疫抑制治疗,在2个月内获得缓解。ITP在达到正常因子VIII水平后发生,静脉注射免疫球蛋白进行治疗。化疗开始前检测到中性粒细胞减少,并使用粒细胞集落刺激因子进行治疗。结论:这些独特的挑战所提出的副肿瘤血液学综合征需要敏锐的临床判断和多学科合作的有效管理。
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"The Immune Conundrum": Acquired Hemophilia A, Immune Thrombocytopenia, and Neutropenia in a Patient with Pancreatic Cancer.

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.

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