Budd-Chiari and ET

No, no, not Bud and chianti! It's Budd-Chiari!

In my last post, I talked about acquired von Willebrand syndrome in ET patients. Here's another acquired condition that ET patients are at somewhat higher risk for: Budd-Chiari syndrome (BCS)

Few ET patients get Budd-Chiari syndrome, but a large percentage of BCS patients do have an MPN, usually polycythemia vera, our sister disease, but a fair percentage also have ET. Because BCS is serious in patients who do develop it, it's good to know about. Treating your ET as directed by your doctor is your best defense against BCS, so if you already know you have ET and are following doctor's orders, you have a better chance of not developing BCS.

BCS is caused when the veins that flow to the liver become blocked, in our case due to our many platelets and tendency to clot. This is serious because it can cause the liver to shut down and can be fatal if not treated.

Patients with BCS may have upper abdominal pain, fluid in the abdominal cavity, or an enlarged liver that shows up on an ultrasound. Blood tests can help confirm BCS, and an MRI can show doctors where the blood flow to and from the liver is blocked. Heparin or warfarin, two common types of blood thinners, might be used to dissolve blockages to the liver. Sometimes stents are used.

There's probably more than you want to know in this 2004 New England Journal of Medicine article about BCS. But the pictures and diagrams are cool!

Be well!

Related: Von Willebrand disease and ET

Related: EM and the weekly poll

(This post covers another oddball condition associated with ET, erythromelalgia)

Home