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“免疫难题”:一名胰腺癌患者并发获得性血友病A、免疫性血小板减少症和中性粒细胞减少症

"The Immune Conundrum": Acquired Hemophilia A, Immune Thrombocytopenia, and Neutropenia in a Patient with Pancreatic Cancer.

作者信息

Geethakumari Praveen Ramakrishnan, Sama Ashwin, Caro Jaime G, Yeo Charles J, Nagalla Srikanth

机构信息

Department of Hematology and Medical Oncology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania.

Department of Medical Oncology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania.

出版信息

Case Rep Pancreat Cancer. 2016 Jan 1;2(1):14-18. doi: 10.1089/crpc.2015.29011.prg. eCollection 2016.

DOI:10.1089/crpc.2015.29011.prg
PMID:30631809
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6319692/
Abstract

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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b43/6319692/a11a29408f9b/fig-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b43/6319692/a11a29408f9b/fig-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b43/6319692/a11a29408f9b/fig-1.jpg

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引用本文的文献

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本文引用的文献

1
Acquired hemophilia A: emerging treatment options.获得性血友病A:新出现的治疗选择。
J Blood Med. 2015 May 8;6:143-50. doi: 10.2147/JBM.S77332. eCollection 2015.
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Successful treatment of fulminant postoperative bleeding due to acquired haemophilia.成功治疗获得性血友病所致的术后暴发性出血。
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Localised prostate cancer and hemophilia A (AHA): Case report and management of the disease.局限性前列腺癌与甲型血友病(AHA):病例报告及疾病管理
Arch Ital Urol Androl. 2014 Sep 30;86(3):227-8. doi: 10.4081/aiua.2014.3.227.
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Acquired hemophilia A: a frequently overlooked autoimmune hemorrhagic disorder.获得性血友病 A:一种常被忽视的自身免疫性出血性疾病。
J Immunol Res. 2014;2014:320674. doi: 10.1155/2014/320674. Epub 2014 Mar 24.
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Severe postoperative hemorrhage caused by antibody-mediated coagulation factor deficiencies: report of two cases.抗体介导的凝血因子缺乏导致的严重术后出血:两例报告
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How I manage patients with acquired haemophilia A.如何管理获得性血友病 A 患者。
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Acquired hemophilia A caused by factor VIII inhibitors: report of a case.获得性血友病 A 由因子 VIII 抑制剂引起:病例报告。
Surg Today. 2013 Jun;43(6):670-4. doi: 10.1007/s00595-012-0290-x. Epub 2012 Aug 14.
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Management of postoperative hemorrhage associated with factor VIII inhibitor: report of a case.VIII 因子抑制剂相关术后出血的处理:病例报告。
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Rituximab and intravenous immunoglobulin (IVIG) for the management of acquired factor VIII inhibitor in multiple myeloma: case report and review of literature.利妥昔单抗和静脉注射免疫球蛋白(IVIG)治疗多发性骨髓瘤获得性因子 VIII 抑制剂:病例报告及文献复习。
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Deaths associated with acquired haemophilia in France from 2000 to 2009: multiple cause analysis for best care strategies.2000 年至 2009 年法国获得性血友病相关死亡病例:最佳治疗策略的多病因分析。
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