JAK2 negative. No further testing ordered

Hi,

New to the forum. Long term migraine sufferer. About a year ago I started experiencing lightheadedness and balance issues so much so that I felt like I was about to faint on a number of occasions. Blood tests revealed my platelets were slightly raised (467). Since then I’ve had numerous tests, MRIs etc (MS was considered) with no real results other than my neurologist diagnosing me with Erythromelalgia (burning red soles of feet, hands etc) Due to this and raised platelets she ordered a JAK2 test which came back normal. Her letter to my GP just says if platelet number increases dramatically over next few years then I could be seen by a haematologist.

My question is should I be pushing for further tests or just leave it at that?

I understand from reading that you can have Essential Thrombocythemia, for example, with JAK2, CALR, MPL or none of these mutations.

The cynic in me always thinks cost plays a part as to whether you get full testing!

Thanks

David

1 Like

Hi David, how many platelet tests have you had so far?

my daughter is 2.5 and had high platelets since found randomly at 8m old. we have been testing every 3 months and they are always high,spiking when ill (to 1,200 as an example) but definitely still high when well. the last year more stable at around 575-590.

She was also negative for all 3 tests you mentioned. (though in kids it seems this scenario is more common even if they later find they have Essential thrombocythemia (ET))

She was at the low end for some iron results so been on iron for a year which the iron score improved but not the platelets. She was passing out though while the iron was low, so have you had that checked?

Other tests they have run on her at various points to help explore/rule out reactive causes are

ultrasound on spleen and liver

Coeliac tests (wheat allergy as IBS can cause high platelets)

Looking for inflammation markers (ie arthritis)

A creatine kinase test which seems to check muscle wastage but wasn’t fully explained

Acute myeloid leukaemia (AML) test again not fully explained but negative

There are some inherited things apparently that can cause high platelets which I used work insurance to test for a year ago and was also negative.it was a gene test of some kind.

For the passing out they did a few ECGs the day of the episodes,

After a few happened we were able to push (hard) for a 24 hour holter monitor. This found a heart pause of 2seconds and aligns with a type of fainting for her age group. in adults the call her type vasovagal syncope/cardio syncope. there is another similar condition called POTS. a common trigger for all is also dehydration.

She had an EEG which ruled out epilepsy

She has not had any MRI although apparently NICE recommends this after 2 passing out or seizure like episodes

No bone marrow biopsy as they say she is too young, but this is apparently needed for an Myeloproliferative neoplasms (MPN) diagnosis.

We have been advised for platelets testing to only test when well fed and watered as dehydration can raise platelets as can any common illness ie cold, flu, covid, norovirus etc. a vaccination could also spike it.

Given the narrow number of causes for high platelets, I have become frustrated at the long delay in getting answers.

The WHO have a flow chart signposting the testing to do with higk platelets that you might find useful? annoyingly it doesn’t come with a timeline for Dr’s to follow!

Been told our daughter is too young for a bone marrow biopsy.

I emailed an Myeloproliferative neoplasms (MPN) specialist (just using google, not a paid thing!) and asked for an opinion on it all after a year and the triple negative results, she suggested I push for a haematologist which I did by asking our GP to arrange it. I have had to be quite pushy and proactive! With the passing of time people seem a bit more willing to do things…!

Anyway at our last appointment the haematologist acknowledged it doesn’t seem to be reactive, so I said ok how do we learn what it is. he agreed to redo the 3 mutations, explaining that as these are acquired it can be sensible to repeat after a period of time to see if one appears. so that is finally happening at the end of the month.

have you had many high results?

have you had any different tests to us?

I have been finding it difficult, especially the general lack of speed to get a diagnosis!

3 Likes

Hi LoisMum,

Thank you for the reply. Firstly I too have a 2.5 year old who is never away from hospital for various tests so I know how worrying and frustrating that can be!

I didn’t know what a platelet was until last June when I visited GP about my Migraines getting worse. I think I’ve had 5 blood tests in the year, not sure how many tested platelets. I’ve been told they ranged from 467 to 430 at lowest, so just in normal range.

What is most frustrating is that depending on what specialist I talked too, there was either no concern re platelet numbers all the way up to yes this needs to be investigated.

Like your daughter I had the 24 hr heart monitor Mine was fine. I had a chest xray, again normal.

My last Neurology appointment when she diagnosed Erythromelalgia, she said that I should be seen by haematology regardless of JAK2 result.

Now JAK2 is negative my last letter just says leave it just now.

I guess it’s just the flip flopping and no real answers that’s hardest to take!

Thanks

David

1 Like

Hello David, I got diagnosed two weeks ago after months of tests, my JAK 2 was negative as well as bcr/abl not detected but my platelets rose to around 487 and I have a massive spleen. I have had a bone marrow biopsy and no fibrosis was found. I will have to have another in a few months as my hematologist is convinced it’ll show some fibrosis. I’ve been put on Iron supplements, aspirin, Hydrea chemotherapy tablets aswell as feburic. Although my bone marrow biopsy was clear of lymphoma and leukemia, it did show presence of irregular megakaryocytes, thrombocytosis and anemia were on my blood results. There were some other factors on my blood results which he said put me in the high risk category the differential count on my cbc monocytes 7, myelocytes 1, juvenile 3 finally LDH was 667.

I’m 44 and I suffer from migraines, bone/muscle aches in my arms Legs, lower-back as well as left sided stomach pain from the spleen enlargement. I also experienced itching and have mild heptomegly.

I was told to be careful in the sun and wear sun cream and stay in the shade as much as possible. I live and work in a very hot country, so that will be tricky!

Anyway I do hope you get answers and maybe push to be retested in a few weeks.

Take care

Louise

@Loismum I do hope you get answers soon!

2 Likes

Hi Louise,

Thanks so much for your response. I think your story pretty much confirms to me that I should be seeking further tests.

Can I ask you about your abdominal pain and enlarged spleen what this feels like? I ask because I’ve developed IBS type symptoms last couple years and I have a stitch like feeling just below left pectoral which I believe is roundabout where the spleen is located.

Thanks again

David

Hello, yes that’s exactly the issue I had. Feeing of fullness and cramps on the left side just under my ribs and also sternum area. I had problems with IBS symptoms a few months beforehand. A ultrasound showed spleen enlargement. I was sent for MRI which showed the size of my spleen and some other details. I couldn’t have contrast dye because I’m allergic to it. I’d say ask your doctor for more tests as you do not feel right.

I hope you get some answers soon.

Take care