Abstract
BackgroundAcquired Hemophilia A (AHA) is a rare bleeding diathesis in patients with no previous personal or family bleeding history. The diagnosis of this disease often delays due to unfamiliarity of physicians with it, which leads to its high mortality rate.Case presentationTwo cases (one 12 years old female and another 18 years old male) were admitted for right upper abdominal mass and right upper abdominal pain respectively at different times. Pre-operative diagnosis of both cases was congenital choledochal cyst. They suffered continuous gastrointestinal bleeding (hematemesis and melena) with reduced hemoglobin to 54 g/L and 60 g/L after Roux-en-Y anastomosis respectively. To investigate the exact bleeding site, Digital subtraction angiography (DSA) of case 1 showed contrast overflow at small branch of proper hepatic artery but had unremarkable result for case 2, whereas gastroscopy of both cases showed unremarkable results. Multiple surgeries were also performed for hemostatic purpose but each time no active bleeding site was found. Finally, hematologists consultation was mandated in both cases and they were diagnosed as acquired haemophilia A. However, unfortunately case 1 patient could not survive because of sever hemorrhage and infection while Case 2 of 18 years old male survived after proper haemophilia treatment catalog.ConclusionAwareness about surgery associated acquired haemophilia A (SAHA) can facilitate quick diagnosis and lifesaving management because the mortality rate in SAHA is high due to lake of knowledge or late recognition of the disease. Bleeding always occurs at surgical sites and it can occur immediately within few hours after surgery in some cases. Hemorrhage may be severe or even life threatening and it presents a special challenge for diagnosis and treatment in a patient who has just undergone a surgical procedure. The treatment strategies for AHA include resumption of hemostasis with either recombinant porcine factor VIII (rpFVIII) or bypassing agents and immunosuppressive therapy to suppress the production of the factor VIII inhibitor.
Highlights
Acquired Hemophilia A (AHA) is a rare bleeding diathesis in patients with no previous personal or family bleeding history
The treatment strategies for AHA include resumption of hemostasis with either recombinant porcine factor VIII or bypassing agents and immunosuppressive therapy to suppress the production of the factor VIII inhibitor
The results showed activated partial thromboplastin time (aPTT) of 105.1 s, FVIII activity 11.2%, FIX activity 119.2%, the titer of factor VIII inhibitor level was 16 Bethesda unit (BU)/ml, so it was concluded that the reason of the intractable bleeding was surgery associated acquired haemophilia A (SAHA)
Summary
We reported two cases of SAHA which were diagnosed after multiple surgeries, the SAHA was recognized by its general characteristics of having continues bleeding, high aPTT, low factor VIII activity, high level of factor VIII inhibitors and lack of previous personal and family bleeding history. The mortality rate in SAHA is high due to the deficient awareness or late recognition of the disease. It requires prompt diagnosis on the basis of SAHA’s specific diagnostic tests and growing awareness of physicians of various specialties. A two-pronged approach including resumption of hemostasis and eradication of factor VIII inhibitors are required for excellent treatment of SAHA
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