Wednesday, 20 August 2014

Brief Repository Update

Colin the Caiman
I received an email from Dr Tobin at the Mayo Clinic recently to update me on their CLIPPERS Respository project. This is a systematic approach to CLIPPERS research which involves gathering as much information about patients as possible in one place to enable detailed research. If you have a diagnosis of CLIPPERS and want to take part, you can read more on the CLIPPERS Repository page.

Anyway, they are currently focussing on patients who had tissue samples available and they have 12 enrolled to date which they are very pleased with. This data is enabling them to take a more comprehensive look at the common features of the condition across different people with less doubt about the possibilities of other diseases being present. There should be some more news about this in the near future.

They are also still very much interested in recruiting people with a CLIPPERS diagnosis but who didn't have tissue samples taken. These people (like me) are still a valuable resource and will form a larger cohort which can be used in the next phase of the research. I have signed up and will update you about the process when I get my enrolment kit.

Apparently, some other laboratories are talking to the Mayo about the possibility of performing different analyses on some of the samples. I think this is particularly exciting as it means there may be a competitive element which will help drive the research. I am hopeful that with this kind of approach some progress can be made in finding out more about what CLIPPERS really is, and how to best treat it.

Read other articles in this series at Living With CLIPPERS.

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Saturday, 9 August 2014

More Medical Ponderings on CLIPPERS

Another paper from Dr Taieb appeared recently, again pondering on the relationship of CLIPPERS to other central nervous system disorders. In this short letter, "A central nervous system B-cell lymphoma arising two years after initial diagnosis of CLIPPERS", he considers a new case where an initial diagnosis of CLIPPERS was made which was based on tests including brain biopsy. Eighteen months later the patient relapsed whilst still taking Prednisolone and a diagnosis of presumed central nervous system B-cell lymphoma was made.
These cases are always worrying for those of us being treated for CLIPPERS. I guess the diagnosis had to change as the patient was no longer "responsive to steroids" which is required for CLIPPERS. But as ever, the question is, was this a simple case of mis-diagnosis under difficult conditions (not least as Lymphoma can also respond to steroids initially) or was it suggestive of CLIPPERS progressing to something else? Dr Taieb considers both these scenarios as possible and doesn't offer an opinion about which scenario he thinks most likely. It is also worth mentioning that Dr Taieb says that this case is one patient out of twelve studied in the 2012 French CLIPPERS cohort, the rest of whom (presumably) retained their original CLIPPERS diagnosis.

In other news, my shingles rash didn't get any worse and has largely cleared up. I have no idea if seven days of Acyclovir five times a day helped or not, but the rash stayed fairly localised. From what I have read, this was pretty mild for shingles as any discomfort stayed at the level of an annoying ache.

Read other articles in this series at Living With CLIPPERS.

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Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

Saturday, 19 July 2014

CLIPPERS and Grade 1 Lymphomatoid Granulomatosis

In May, I reported on a curious case of CLIPPERS which involved skin lesions. There have now been two follow-up letters discussing this case. In the first, Dr Taieb (who has also published CLIPPERS-related papers) suggests that CLIPPERS could be a manifestation of a grade 1 Lymphomatoid Granulomatosis which is thought to be a pre-lymphoma condition. The key comment (from my reading) is that grade 1 Lymphomatoid Granulomatosis and CLIPPERS may be indistinguishable in terms of diagnosis and treatment response. In addition,  grade 1 Lymphomatoid Granulomatosis "does not necessarily progress to grades II or III" which would presumably explain why there are so many "stable" cases of CLIPPERS out there.
In response to this, the original author, Dr Kossard suggests this association is premature. My interpretation of the letters is that it is hard to be sure, even from studying tissue samples, about any possible relationship between CLIPPERS and Lymphomatoid Granulomatosis. It does seem, though, that the net is very gradually tightening around CLIPPERS in terms of figuring out exactly what it is and where it sits in relation to other rare conditions.

In other not-so-exciting news, I came out in a painful rash on my face which I initially thought was caused by  insect bites but got gradually larger. I was quite surprised to be told by my doctor I had shingles. Of course, being immune-suppressed, means that despite this rash being fairly small on the scale of shingles, no chances are being taken. So now I am taking anti-viral medication five-times a day as well as anti-viral ointment for my eye (as the rash is closer than it looks on the picture).
Read other articles in this series at Living With CLIPPERS.

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Monday, 7 July 2014

News Update

About 3 weeks ago, I had my first brain-scan of 2014, this time at the Institute of Neurology in London. I did ask my neurologist whether they were going to try anything different, as the IoN scanners are more research-oriented, but he told me they were just spreading the load of clinical imaging cases. It's funny, that having scans less often is in some ways more stressful because you wonder what might have been going on in the interim. Anyway, I have now had the unofficial feedback on the scans which was that:

"Everything is good. There was a tiny bit of signal change in the pons, as before and no enhancement. No change from last year."

So this is good news, especially as I have now been off Prednisolone for two years. Sounds like a bit of residual CLIPPERS-related damage which I will have to put up with, but I can't associate that with anything specific in day-to-day life.

Read other articles in this series at Living With CLIPPERS.

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