portal Hypertension

[DarcyShepp]

Hi everyone,

Has anyone had portal hypertension due to blood clots caused by an MPN?

If so, I’d really like to hear your experience.

Thanks in advance

[Scaredy_cat]

About 25 years ago I was diagnosed with a portal vein thrombosis and enlarged spleen. They suspected something possibly wrong as the cause but eventually my blood settled back to normal. It wasn't till about 10-15 years ago they diagnosed ET and by then the genetic testing had come in and I had Jac2. The spleen remained enlarged. I continue to have 3 yearly endoscopy to check for varices at the bottom of my oesophagus which can be a symptom of the portal vein thrombosis

[DarcyShepp in reply to Scaredy_cat]

Thank you so much for your reply, and I am sorry to hear that you suffered with this. Unfortunately I have varices already, I am having an endoscopy to determine what stage they are at but I know they are in my esophagus, abdomen etc. My spleen is still enlarged and I have been told it’s down to the portal hypertension and not the mpn. I’m not sure which is better tbh, it all a bit scary 😱

[LPPL]

hi, about 18 yrs ago was diagnosed with portal vein thrombosis, which has caused portal hypertension. Initially as no one could figure out why I’d got the blood clot ( I was 26yr) I was simply discharged with no monitoring or meds. After a few years had a massive bleeding event I was lucky to survive, due to oesophageal varices. Then they discovered ET, Jak 2+. I now take propranolol to help keep portal pressure low, but I’ve recently developed more varices after another (smv) clotting incident.

[DarcyShepp in reply to LPPL]

Hi LPPL, I am so sorry to hear you had another clot. You must be around my age (I’m 42) Thank goodness you survived. How are you feeling now? I have had the most awful stomach pains for portal hypertension but they have put me on beta blockers which seems to be reducing the symptoms. I’ve been told in the future a possible liver transplant can cure this, had you heard the same? There are also some other treatments they can offer and my blood thinners are being changed to Warfarin as the effects can be reversed faster than appixaban, should you bleed. I am not sure if I should be more worried about this or the secondary MF.

They didn’t pick my jak 2 mutation up after my clotting incident either which is frustrating to say the least.

Keep in touch and let me know how things progress

[user2021a]

I have Budd Chiari- check out BLT website for further info- the veins in my liver all clotted and had portal hypertension and a variceal bleed as a result. This caused by PV which is an MPN. TIPS shunt saved my life when first diagnosed and again after bleed (still keep having clots)

[sinb]

I have been diagnosed a year ago with portal hypertension due to a 4mm blood clot in the portal vein. Numerous vein collaterals have been developing in the liver. Also there is clotting in the splenic vein, more collaterals and varices in the stomach. Fortunately, I have not had any bleeding episodes. The hematologist, a MPN specialist and GI specialists have not concurred on the use of anti coagulants and anti platelet meds. They are considering a stent for the portal vein followed with warfarin for 3 mos. I have an appt. with the IR specialist at UCSF in October to determine if the stent would be the approach to take with the risks of having bleeding during the procedure due to the numerous collaterals. The spleen is very enlarged. Blood clots are the major issues for all the MPS which is why aspirin is used at the onset of diagnosis. I did not know how advanced my condition is since the clotting has been asymptomatic. Endoscopies and abdominal CT scans discovered this progression of PV which was diagnosed seven years ago but had been a slow burning issue for more years than I can figure. I was advised to stay active, don't change my lifestyle and continue with daily hydroxyurea and carvedilol(beta blocker). I will need the pro and cons about the stent and determine if that is the best approach. My advice to all MPNers is to have abdominal CT scans done soon after a dx so one can monitor the disease progression and can make choices based on evidence. Best to you and your decisions.

[DarcyShepp in reply to sinb]

Hi Sinb,

Thank you for sharing your experience. It sounds like we’re in the same boat. I had splenic vein thrombosis, portal vein thrombosis and partial mesenic vein thrombosis 3 years ago. My spleen was enlarged then and has enlarged even more since, the specialist has said it’s filling with blood. The beta blockers are helping my symptoms. They are going to do another endoscopy to see what grade the varices are then they will decide how to treat them.

Good luck with your treatment.

Just out of interest, except from PV, was any indication given as to what made the clots form at the time they did?

My PV was undiagnosed at the time so I was taking iron tablets for anemia and I was on the contraceptive pill and these are the only external factors that could have made them form at that specific time.

Thanks

[sinb in reply to DarcyShepp]

The clots are labeled as chronic, so timewise is a mystery as to when it started. Ultrasounds of the gall bladder area was done just as the PV was dx. But no mention of clots in the liver area. That being seven years ago, I feel the increase in formations may have resulted from ramping up of the numbers of White Blood Cells and the levels of daily inflammation due to pruritis, stemming from the Jak2. I did aspirin for the first year but had horrible stomach pains, so discontinued taking it. Now curious as to the effects of a Covid infection which I had without vaccines and evidences of the rise in clots in the population. And a warning with contraceptive use is thrombosis. How long were you on it? Meanwhile, we stay vigilant.

[Dodders]

I had a portal vein thrombosis, and as a consequence upper gastric varices which ruptured on several occasions. It would seem that such events are the most significant problem related to the thrombosis. A TIPS procedure was mentioned but not done. I was prescribed a beta blocker- carvedilol- to reduce the localised hypertension causing the varices. That's about all I can say/remember about the thrombotic issue you have. Good luck going forward.

[DarcyShepp in reply to Dodders]

Thank you so much for sharing your experience Dodders, do you mind if I ask, was that along time ago and have you had any issues since you started taking the carvedilol?

It must have been very scary when they ruptured. I am taking the same treatment for this issue.

Thanks in advance

[Raineless]

Yes I had some major clots in my portal vein and spleen. Causing the blood to flow backwards and fill my stomach and bowels with blood. I'm now on apixiban blood thinners and hydroxyura. Strange part was when I went to the emergency my platelet counts were in the 450-480 range and that's the lowest I've seen them in the past 10 years they are in usually well over 800.

[DarcyShepp in reply to Raineless]

Hi Raineless, thanks for your reply!

That’s so interesting, thank you for sharing.

My bloods were all normal, and still are but I suffered those big clots.

Nobody seems to understand why.

Have the blood thinners sorted your issues out?

[Raineless]

I'm waiting for the results of my last CT scan then I'll know if it's improving. I'll update you. My next appointment is on Aug 30th I should have the results by then

[DarcyShepp in reply to Raineless]

Hello again,

It might be worth asking if beta blockers can help with your condition. I wasn’t put on any after my clotting episode but 3 and a half years later a new specialist said I should have been put on them in the months following as they may have helped to reduce the clot. I’m on them now and have been on appixiban ever since but I’ve been told the clots are like concrete now and it’s unlikely that they will be able to reduce the size of them.

Just a heads up but I’m sure your specialist has it all under control.