PV with chorea

[Crotchety]

Crotchety.

Anyone else with this ? I'm female, over 70, PVjak2 positive on aspirin, hydroxicarbamide for 7 years. Sudden new rare symptom of r.side involuntary movement. .Haematacrit 45/6 . Can't drive. Alarming.

[Manouche]

I am sorry to hear about your chorea. This is a rare PV symptom but well documented . You should get much better very soon. ncbi.nlm.nih.gov/pmc/articl...

[john0084 in reply to Manouche]

Many thanks Manouche you are clever, I learnt a new symptom of pv.

[mhos61]

I’m sorry to hear this Crotchety. As you say, it’s very alarming. I have never heard of this side effect.

I’ve just browsed through the attachment from Manouche which is interesting. What has your haematologist proposed? Maybe an increase in hydrea or alternative medication may help??

[Crotchety in reply to mhos61]

Thank you all for replying. My family had asked for a private referral to a PV specialist even before the chorea. Jakavi seems a possibility, but not licenced in UK for PV. I am very breathless,losing weight, night sweats, v tired. All much worse in the 2 months before the sudden onset chorea. If blood count ok even if only just in upper limits noone interested insymptoms. Hope the specialist will see me even if no MF. Otherwise ? Crotchety.

[mhos61 in reply to Crotchety]

You are clearly symptomatic with new changes, your haematologist should be interested!

I hope you get an appointment with an MPN Specialist soon. Many PV patients have had an excellent response to Pegasys.

Your GP can refer you to an (NHS) MPN Specialist, it is your right to get a second opinion. Professor Claire Harrison at Guys and St Thomas in London is a leading MPN Specialist, as is Professor Tim Somervaille at The Christie, Manchester. I don’t know what the waiting list is like though.

You could also ask your appointed haematologist is it possible to liaise with an MPN Specialist to discuss your case.

Thoughts are with you. Keep us updated.

Mary

[Bluetop]

I had never heard of this and it must have been so alarming the first time. I too am nearly 70 with PV taking asprin and hydroxy. As Mary says, I am sure your GP should be able to refer you to a specialist. Let us know how you get on and best wishes.

[PFR1937]

Hi Crotchety. My mother is 85 and has had PV (Jak2 Positive) for over 10 years.She takes Hydroxycarbamide (500mg - 9 tablets / week) and Rivaroxaban (1x10mg tablet each day) for the condition. Her spleen is enlarged to its maximum. She has often suffered from tiredness and shortness of breath, occasional dizzy spells and visual disturbances. We know she has a blood clot behind one eye. A year ago she suffered cystitis and a soreness of the mouth and tongue inhibiting her speech slightly and what I would call a restless leg type syndrome whereby she found it difficult to get comfortable and stay still. The last time I posted on this forum it was about restless legs.

After a visit to her heamatologist and some blood tests, her results were all within 'acceptable' limits but the haematologist did agree to a reduction from 10 to 9, 500mg tablets / week of Hydroxycarbamide as she was potentially becoming a little anaemic. Her symptoms then generally improved although she still suffers with visual disturbances and balance problems occasionally.

Just recently her symptoms have come back (except the cystitis) and now I think I can say that the restless legs are more like chorea in that she has involuntary movement of her legs and arms (except when sleeping), a serious lack of balance making it difficult to walk unaided and she keeps biting her tongue, slurring her speech and losing concentration. Her Haematologist was concerned enough to send us to A&E last week to get her an MRI head scan and seemed to be putting the problem down to a neurological cause (possibly related to an acoustic neuroma she had been treated for a few years ago). The scan was fine and showed no specific problems so we are still in the dark as to the reason for the recent chorea etc.

I too was about to ask for a second opinion via an MPN specialist so I would be interested to know how you got on with yours - did you manage to resolve your problem? Was it related to the HU drug?

Any information would be gratefully received - thanks.

[Crotchety in reply to PFR1937]

Hi. PFR1937. re Chorea. I still have it. It is r arm, r leg and when bad a slightly swollen feeling in the r side of the lip. My grandchildren call my arm/ hand Cheeky Charley. It is worse when I am tired or excited. If I'm using the R arm or leg I have no problem. However if sitting passively, my R arm, for instance, plays every stroke of every tennis match I view on TV and responds to every thriller though the left side is silent. I am L handed mainly. It has its funny side though its constant activity when resting can be exhausting. Apparently it is quiet when I'm sleeping. My R leg gets very uncomfortable sitting or sleeping if I have been walking which I like doing but suspect that is old age.

The neurologist thinks it is PV related and offered me pills which I declined. The MPN specialist at UCH thought it was a small vein problem but it hasn't shown as such. I was very distressed and embarrassed when it first happened but it has calmed down though still noticeable. I am on 10 500 mg tablets of hydroxicarbamide a week and though I still have night sweats and get very tired after doing what use to be not too much . I do most things. I do get very short of breath - not asthma or my heart apparently so probably PV. I now can't play more that one game of badminton which is really annoying. I am sorry I can't be more help.

[PFR1937 in reply to Crotchety]

Hi Crotchety. Thanks for your reply and that information is very helpful in itself. Strange that yours is all on one side - I wonder why that is? My mum also gets very tired and short of breath but as you say maybe some of that is old age.I anticipate that we are going to bounce between neurology and haematology departments in an attempt to resolve this but I will let you know how we get on in case it is of any use to you / others. Take care and keep up the badminton.

[PFR1937]

Hi Crotchety. A quick update.We have now had two appointments with a consultant neurologist. He prescribed a sedative called Clonazepam which belongs to a group of medicines called benzodiazepines. It's used to control seizures or fits due to epilepsy, involuntary muscle spasms, panic disorder and sometimes restless legs syndrome. She was started on half a tablet a day now increased to one tablet per day. We were made aware that this is solely a drug to control the symptom (chorea) not the cause, which requires further investigation and for which they have no clues at present. The drug has definitely reduced the involuntary movements considerably and reduced the tongue biting which was probably the most annoying thing.

I have found evidence of a case of chorea being reported in a patient who didn't know they had PV but once the patient was diagnosed and treated with Hydroxyurea the chorea went away. So there is a link (with your experience too) but more than that I can't say. They have offered us a test for Huntingdons Disease which is due to a gene fault so we can eliminate that as a cause. Blood test results don't show any cause for concern either.

My guess is that the chorea is due to the PV and an MRI scan of the brain has shown some evidence of small vessel disease which you mention too.

I will let you know if / when we get to the bottom of it. I hope you are doing well. All the best.