61-year old patient diagnosed with mild Hemophilia A, treated by plasma derived concentrate in on-demand regimen presented with abdominal pain and vomitus of gastric content. Laboratory tests revealed FVIII-function: 0.002 IU/ml. Abdominal CAT scan ruled out acute pancreatitis, but revealed irregular, locally circular thrombus, 2-5 mm thick, virtually in the entire lenght of the abdominal aorta.
What approach would You consider in management of patient?
Antiplatelet treatment? Antithrombotic treatment? Would you continue factor VIII replacement treatment ? What dosage of mentioned treatment would you administer?
Dear Peter,
thank you for posting this very interesting case. Would you be so kind to add some other informations, like these: cardiac rhythm; blood lipids; family history of CAD or MI; personal history of hypertension; concomitant treatments; and symptoms, if any, related to aortic thrombosis (i.e., abdominal angina or peripheral embolism)
thank you
Hi Peter,
Very interesting case indeed. TFPI may be tried in such cases. the dosage of TFPI may be calibrated based on the degree of thrombosis.
Omer
This is a very interesting case. My curiosity is ... is the thrombus related to FVIII concentrate treatment?
Very interesting case indeed. Personally, to rule out the possibility of having thrombus formation due to FVIII concentrate, I would monitor pre and post-treatment FVIII levels and adjust accordingly. Also, the FVIII-function result of 0.002 IU/ml is past the thrombotic event or before?
Dear Peter,
The case is very curious; we have thus no evidence supporting any therapy.
I would test this patient for thrombophilia including sticky platelet syndrome, antiphospholipid syndrome, myeloproliferative disorders, and paroxysmal nocturnal hemoglobinuria, which can be associated with arterial thrombosis. I would also make a comprehensive screening for cancer.
I would prefer the intermediate dose of LMWH as an initial therapy and I would continue with this therapy until thrombus resolution. The decision about the factor VIII replacement depends on frequency and severity of bleeding complications. If factor VIII replacement would be regular, full therapeutic dose of LMWH could be required.
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