| Abstrakt: |
• Acquired hemophilia A (AHA) rarely presents as paraneoplastic syndrome. • We report a case of AHA in periampullary carcinoma. • Recombinant factor VII, cyclophosphamide, steroids, FEIBA, Emicizumab can be used for management. • Removal of primary cause sometimes cause resolution of disease. Acquired hemophilia A (AHA) is a rare disorder characterized by development of antibodies against factor VIII, which can present as paraneoplastic syndrome in various malignancies like periampullary cancer, cancer of lung, prostate, gastrointestinal stromal tumour and non malignant cases like pregnancy, autoimmune disease and medication. We report a case of elderly man presented with paraneoplastic AHA in periampullary carcinoma in preoperative period which was diagnose by mixing study and inhibitor assay and managed with bypass agents like recombinant factor VII, FEIBA and immunosuppresion to eliminate inhibitor with help of steroid, cyclophosphamide and emicizumab. Patient underwent Whipple's pancreaticoduodenectomy after which coagulation study became normal in immediate postoperative period. Patient was discharged and followed up with chemotherapy. Periampullary carcinoma presenting as AHA is rare and rarer in pre-operative settings. The usual mode of presentation is bleeding after biopsy and from minor surgical scars. The pathogenesis is yet to be delineated. It is managed by factor VIII administration and immunosuppressive therapy. High index suspicion should be there to diagnose AHA as a paraneoplastic manifestation and elective surgery should be delayed till normalization of coagulation parameters. [ABSTRACT FROM AUTHOR] |
