The second of the recent mini-flurry of CLIPPERS papers returns to the topic of CLIPPERS being mistaken for Primary Central Nervous System Lymphoma (PCNSL), with a short comment and response on the recent case-study by De Graaf et al where a patient fulfilling the diagnostic criteria for CLIPPERS went on to develop PCNSL. There is some discussion which I don't understand (as I am not a doctor) about the time-course of tests and patient response in this case. The advice from both sets of authors is that patients newly diagnosed with possible CLIPPERS should be closely followed, especially for the first 12 months, in case there is another cause. This comes back to the lack of a definitive diagnostic test for CLIPPERS, and the fact that ultimately the diagnosis is by exclusion of other conditions which could also have similar symptoms and MRI appearance. I was told in 2011 there was an outside chance I could have PCNSL rather than CLIPPERS - the big problem is that initially PCNSL responds to steroids like CLIPPERS but then returns and is very hard to treat. I spent a nervous few months on steroids before getting to the point where I thought that if it was PCNSL, that it should probably have recurred by now.
In fact, the most controversial part of the new comment is the title: "Should CLIPPERS Be Considered a Prelymphoma State or a New Inflammatory Disease?" From my understanding (doctor disclaimer again) it is pretty clear that whatever the problems presented by CLIPPERS, it is still preferable to PCNSL. While there have been one or two cases reported of PCNSL being mis-diagnosed as CLIPPERS, I'm not aware of much evidence for CLIPPERS being a precursor to something else - but it is quite early days. As there is some variation in CLIPPERS symptoms and response to treatment, it may be that it isn't a single well-defined "condition" but one which differs from person-to-person. In the absence of a definitive test for CLIPPERS this remains a possibility, particularly as there is increasing variability in the published cases regarded as CLIPPERS.
Still lots to do then.
Read other articles in this series at Living With CLIPPERS.
Living With CLIPPERS by Bill Crum is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.
