Successful treatment of refractory pancreatic hemorrhage and pseudoaneurysm related to chronic pancreatitis in a patient with severe‐type hemophilia A: A case report

Pancreatitis is an inflammatory condition and has the nature of auto-digestion and the potential of interstitial hemorrhage, which may cause permanent pancreatic structural changes, pseudocysts and hemorrhage.1 Causes of the chronic pancreatitis are now still unknown. Some patients with chronic pancreatitis have even formation of life-threatening pseudoaneurysm, which incidence is about 1.3% to 10%. The mortality rate of pseudoaneurysm rupturing is up to 40% to 90%.2, 3 Hemophilia A is an inherited bleeding disorder due to deficiency of coagulation factor VIII (FVIII). There have been few reports on hemophiliacs with pancreatic hemorrhage and pseudoaneurysm. In such a condition, it is extremely dangerous for the patient because bleeding may be uncontrollable and lethal. Even if bleeding is controlled, poor tissue healing in hemophiliacs might still cause delayed/recurrent hemorrhage. Here we reported such a troublesome case. A 42-year-old severe-type hemophilia A male patient was diagnosed with chronic pancreatitis in 2014. Severe epigastralgia radiating to the flank and elevated serum amylase and lipase levels occurred intermittently for 2 years. Later, he got the first episode of pancreatic hemorrhage. Hb level dropped 1.7 g/dL and a pseudocyst was found by CT scan. After aggressive FVIII replacement therapy q8h to maintain a trough level of >80%, his condition rapidly recovered. He returned to the routine intermediate-dose prophylactic therapy. Forty days later, pancreatic hemorrhage recurred by repeated CT scan (Figure 1) and Hb level dropped 2.0 g/dL; and a splenic artery pseudoaneurysm was revealed by CT scan and angiography (Figures 1A and 1B). Under aggressive FVIII replacement therapy, we performed super-selective transarterial embolization (TAE) and blocked successfully the pancreatic pseudoaneurysm (Figure 1C). Then, his condition got stabilized. When the rFVIII dose was gradually tapered to every other day (QOD) 1 month after TAE, pancreatic hemorrhage recurred, and repeated CT scans with contrast showed no bleeding from the embolized splenic pseudoaneurysm, which has the similar pattern to his post-TAE status. For the entire year following TAE, replacement therapy was never discontinued as he experienced various amount of pancreatic bleeding every month. Whenever replacement therapy was tapered to QOD under a stabilized condition, he experienced pancreatic hemorrhage again. As per the regulations of the National Health Insurance program of Taiwan, high-dose FVIII prophylactic therapy (50iu/kg QD) is not reimbursed. Fifteen months later, his replacement therapy was difficultly but finally successfully tapered. No recurrent bleeding had been found for 1 year since then. Normal tissue healing requires adequate hemostatic function for an appropriate duration. A clear wound can take up to 4 weeks to heal.4 Poor/delayed wound healing is common in patients with bleeding disorders due to deficiency of fibrin network at the wound for supporting cell proliferation.5 For recurrent pancreatic bleeding target tissue in hemophiliacs, high-dose prophylaxis therapy for 3 to 6 months may be necessary. In conclusion, when pancreatic hemorrhage occurs in hemophiliacs, replacement therapy of adequate dose and treatment period is critical for preventing repeat hemorrhage and promoting healing. TAE could be a well-tolerated procedure for pseudoaneurysm under adequate FVIII support and direct compression on the puncture wound. Because of the rarity of such cases, we believe that our clinical experience can be helpful to clinicians who may encounter similar patients. All authors declare no conflicts of interests.

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