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

Abstract

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.

Unforeseen encounter of acquired hemophilia A in a preoperative case of periampullary carcinoma: A case report

“…Usually AHA is seen in post operative period as seen in literature review by S. Reitter et al in 4 cases of pancreatic cancer [ 4 ]. Similarly Mavroeidis et al reported AHA in postoperative case carcinoma of ampulla of vater after two month of surgery which was attributed to peritoneal metastatic disease which was also confirmed by Geethakumari et al adenocarcinoma of pancreas [ 10 , 11 ]. None of the cases reported in preoperative period as per our knowledge.…”

“…Mavroeidis et al and Geethakumari et al had reported paraneoplastic hemophilia associated with pancreatic cancer [ 10 , 11 ]. We report our experience of another case of paraneoplastic hemophilia associated with periampullary cancer in an elderly patient in preoperative period after thorough investigation and management.…”

A case of acquired hemophilia A after pancreaticoduodenectomy for distal cholangiocarcinoma

“…In certain cases, AHA may develop following major surgery, including pancreatoduodenectomy ( 11 , 12 , 15 ). APCC ( 16 ) and recombinant activated factor VII (NovoSeven ® ) ( 17 ) preparations are typically used in the acute phase of AHA for hemostasis ( 5 ).…”

National survey of hepatobiliary and pancreatic surgery in hemophilia patients in Japan

“…In addition, as these hemophilic patients age, not only hepatic malignancies but also bile duct cancers and pancreatic cancers are developing and requiring surgery if possible 11 . Although some case reports have been published in the abdominal surgical area, no systematic reports have been found in the updated literature 12‐15 …”

“…11 Although some case reports have been published in the abdominal surgical area, no systematic reports have been found in the updated literature. [12][13][14][15] The group of one of the authors reported the results of hepatectomy in hemophilia patients in 2018. 16 The study reported on 10 patients and showed no differences in operative time, blood loss, red blood cell transfusion rate, major complication rate, and mortality rate.…”