An improved understanding of cutaneous T-cell lymphoma

Wednesday 28 September 2022

September 2022 is unlikely to go down in our collective memories with its nomination as Blood Cancer Awareness month being a main feature, but nonetheless it has been. Lymphoma Action, one of the main patient supporting charities for lymphomas in the UK, has been running a social media campaign to highlight the breadth of symptoms that lymphoma can present with, often hugely challenging in both their range and limited specificity.

For the clinical oncologist, lymphoma itself is a fascinating twist and turn of a tumour group, with their seemingly being no limit to the patient characteristics, presentation and indeed sub-type of disease one can encounter. This variety was one of the attractions of the subspecialty, offering more certainty that I would not be spending decades of a career contouring and making plans for a single organ or body region, but with that comes the exchange of spending time of often being a bit unsure and having to do a lot of thinking and checking in with colleagues.

There are some subtypes of lymphoma that are even more challenging than most, and I would categorise primary skin lymphomas in that group, especially cutaneous T-cell lymphoma (CTCL).

They are by any definition rare or at least uncommon, their presentation is vague and at early stages easily confused with the fantastically more common benign dermatoses, and the histopathology is often challenging especially without full ability to apply really good clinico-pathological correlation, which frequently requires the passage of time. These are probably some of the main factors at play when PROCLIPI (a PROspective Cutaneous Lymphoma International Prognostic Index study) and other observational studies show that patients can have apparent diagnostic delays stretching into years.

We also know that one primary skin lymphoma is not equal to another, although we don’t always know why (PROCLIPI may eventually iron this out further), and patients with very similar looking diseases can have a course that ranges from the indolent nuisance to the life threatening over a time period. Equally, the patients themselves are often fascinating in their variation of the degree of symptoms and distress they experience. I have had true stoics who I have had to train myself not to flinch at the site of their extensive swathes of painful looking tumours and ulcerating plaques across huge amounts of their bodies, and people with tiny quantities of eczema like disease who feel real despair when they look at themselves.

Humans are not very good at looking past the skin-deep, and it is no surprise that there is incredible psychological burden with a disease that one is reminded of every time there is a glance in the mirror, or a reflex grimace of a stranger. There is a lot of research into the quality of life (QoL) and psychology of patients, including the development of assessment tools that not only make it easier (in theory) to compare degree of severity over time and between observers, including skin factors and functional factors. Sadly despite the preponderance of evidence that patients with CTCL suffer high levels of distress and have wide ranging needs, certainly locally to me, the ability to access specialist psychological support or even a clinical nurse specialist (CNS) is very constrained in the overall scheme of noisier and more numerous patient groups.

Some regions of the UK are a little better served and have a higher volume of patients across a wide or more populous catchment, and each super regional multidisciplinary team (MDT) should have access to the required variety of clinical decision-makers, treating teams and diagnosticians. It is not always the case though that our patients find it easy to travel, and developing and maintaining more regional services is really important. Upskilling clinicians is a real challenge and asking busy teams to subdivide up their CPD time or even brain space for small numbers of patients can feel tough.

Fortuitously the past couple of terrible years have actually had a number of high points for CTCL with one being the proper adoption of ICT and remote working for our trusts (I can finally reliably join our super-regional MDT without having to battle with a baffling variety of cables, codes and systems!). Additionally, online educational events have expanded and been much more accessible for some, and I include in that the excellent RCR skin and extranodal lymphoma course, which featured some real expertise and shared first-hand knowledge. Further, access to systemic and topical therapies after some successful applications to NICE, supported by expert opinion and the UK Cutaneous Lymphoma Group, has helped us claw some of the gains we need to make for our patients especially with advanced disease, as well as having encouraged pharma to support more educational and information sharing events.

In short, I find skin lymphoma really hard but also gripping. The conditions are complex, the patients are so varied, and the group of colleagues I have to lean on to help make the diagnosis, and to get the treatment right (and to chivvy me along when it is not going well) is small but very valuable. The technicality of it all I really enjoy (I still think Total Skin Electron Beam Therapy is cool even just to say), and despite the difficulties of meeting their needs, the chronicity of the disease means the relationships built with patients are rewarding, and I look forward to seeing how they are all doing (albeit at times with bated breath). The community of CTCL clinicians in the UK is fairly tiny, but populated with some real talent, which I don’t count myself in at all, but I am lucky to be able to ask for help when needed.

If you want to know more about skin lymphomas, your local super regional MDT will be able to help you and look out for the Lymphoma Action ‘CTCL Map of Britain’ which is in production currently for those details. The UKCLG 2022 educational meeting is in London on Friday 4 November 2022.

Dr Eleanor James
Consultant Clinical Oncologist, Nottingham University Hospitals NHS Trust


This blog has been supported by Kyowa Kirin Limited.

The skin and extranodal lymphoma education course was organised by the RCR and funded by Kyowa Kirin Limited – and is free to access on the RCR e-learning hub.