Geethakumari Praveen Ramakrishnan, Sama Ashwin, Caro Jaime G, Yeo Charles J, Nagalla Srikanth
Department of Hematology and Medical Oncology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania.
Department of Medical Oncology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania.
Case Rep Pancreat Cancer. 2016 Jan 1;2(1):14-18. doi: 10.1089/crpc.2015.29011.prg. eCollection 2016.
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. 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. These unique challenges posed by paraneoplastic hematological syndromes warrant the need for astute clinical judgment and multidisciplinary collaboration for effective management.
恶性肿瘤相关出血可能带来诊断难题。我们报告了一例胰腺癌患者并发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症的独特病例。一名66岁男性,新诊断为胰腺癌,术前血液学评估正常,因行胰十二指肠切除术被送往手术室。由于胆囊积脓、胆管炎和疾病局部范围,手术中止。术后,患者出现出血倾向,伴有皮肤黏膜和腹腔内出血,活化部分凝血活酶时间延长。评估显示高滴度的VIII因子抑制剂,确诊为AHA。使用重组活化VII因子、VIII因子抑制剂旁路活性等旁路药物以及类固醇、环磷酰胺和利妥昔单抗进行免疫抑制治疗,2个月后病情缓解。在VIII因子水平恢复正常后发生了ITP,采用静脉注射免疫球蛋白进行治疗。在开始化疗前检测到中性粒细胞减少症,采用粒细胞集落刺激因子进行治疗。副肿瘤性血液综合征带来的这些独特挑战需要敏锐的临床判断和多学科协作以进行有效管理。