Acquired Hemophilia in an Elderly Patient with Carcinoma of the Ampulla of Vater

Abstract

KeywordsAcquired hemophilia · Carcinoma of the ampulla of Vater · Paraneoplastic acquired hemophilia Abstract Acquired hemophilia is a rare autoimmune bleeding disorder related to the production of autoantibodies that inhibit clotting factor VIII or IX. The underlying cause can be autoimmune disease, malignancy, pregnancy, or medications, but it is most commonly idiopathic. Here we present the case of an 81-year-old patient with locoregionally relapsed periampullary carcinoma who presented with soft tissue hematoma and an abnormally elevated activated partial thromboplastin time (aPTT) in the presence of a normal prothrombin time. A diagnosis of acquired hemophilia was established. The patient was managed with immunosuppressive prednisone and cyclophosphamide plus immunoglobulin G. He also received a cycle of chemotherapy with gemcitabine and oxaliplatin, because the underlying malignancy was the cause of the bleeding disorder. Care was complicated by neutropenia and nosocomial fever, but the patient eventually showed signs of clinical stability, while the aPTT decreased 2-fold. The patient was successfully discharged from the hospital and continued treatment in outpatient care.

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.…”

“…Desmopressin increases FVIII:C by release of factor VIII and Von Willebrand factor but less effective on repeated administration. If possible, invasive procedure to be avoided otherwise replacement therapy before and after the procedure to be given [ 10 ].…”