Author disclosures Alejandro Ariel Gru is a paid consultant for Seattle Genetics and has an ongoing relationship with Innate Pharma. John Zic is a site principal investigator for clinical trials for products from Elora and Galderma. The other authors report no actual or potential conflicts of interest with regard to this article.
Disclaimer The opinions expressed herein are those of the authors and do not necessarily reflect those of Federal Practitioner, Frontline Medical Communications Inc., the US Government, or any of its agencies. The manufacturers did not provide equipment or other forms of material support.
John Zic. Do you prefer one imaging modality over the other? CT scan with IV contrast vs PET/CT?
Lauren Pinter-Brown. I tend to use PET/CT because it illuminates extranodal sites as well. I have to admit that sometimes it’s a problem to get that approved with insurance.
John Zic. In the federal system, many PET/CT scans are performed at other facilities. That would be an extra step in getting approval.
You mentioned Sézary syndrome. We should consider a diagnosis of Sézary syndrome when you have a patient with erythroderma, which means that they have > 80% of the skin covered in redness and scaling.
Lauren Pinter-Brown. The first step is to do a complete blood count (CBC) and see if there’s a lymphocytosis. Sometimes that really isn’t very sensitive, so my go-to test is flow cytometry. We are looking for an abnormal population of cells that, unlike normal T cells, often lack certain T-cell antigens. The most common would be CD7. We can confirm that this is a clone by T-cell gene rearrangement, and often in Sézary we like to compare the gene rearrangement seen in blood with what might be seen in the skin biopsy to confirm that they’re the same clone.
John Zic. That’s an excellent point. I know there are specific criteria to meet significant blood involvement. That is a topic of conversation among CTCL experts and something that might be changing over the next few years. But I think as it stands right now, having a lymphocytosis or at least an elevated CD4 count along with having a clone in the blood matching the clone in the skin are the first 2 steps in assessing blood involvement. However, the flow cytometry is very important. Not all medical centers are going to do flow cytometry—looking specifically for a drop of the CD7 or CD26 antigen among the CD4 population. But that is one of the major criteria that we look for in those patients with suspected blood involvement.
Marianne Tawa. Additionally, we would advise obtaining flow cytometry on patients that look like they have a robust skin burden with lots of patches, plaques, or tumors. We also perform lactate dehydrogenase (LDH) with staging.
John Zic. What do you usually tell patients with early-stage disease, those that have patches and plaques?
Marianne Tawa. For patients with stage IA disease, we are very optimistic about their prospects. We explain that the likelihood that early-stage disease will progress to a more advanced stage or rare variant is unlikely. This is very much a chronic disease, and the goal is to manage appropriately, palliate symptoms, and preserve quality of life (QOL).
Lauren Pinter-Brown. I often refer to a landmark paper by Youn H. Kim and colleagues that shows us that patients with IA disease who are at least treated usually have a normal lifespan.7 I encourage patients by sharing that data with them.
John Zic. Sean Whittaker and colleagues in the United Kingdom identified 5 risk factors for early-stage patients that may put them at higher risk for progressing: aged > 60 years; having a variant called folliculotropic MF; having palpable lymph nodes even if they’re reactive on biopsy, having plaques, and male sex.8 For staging of lymph nodes, what’s your usual approach when you see a patient with palpable lymph nodes?
