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.
Lauren Pinter-Brown. As you previously discussed, this is an illness of older people, and Vietnam veterans now are in their 60s and 70s. They may account for a lot of these diagnoses.
John Zic. That’s a good point. There’s quite a bit of talk about exposure to Agent Orange. But honestly, we really don’t know the cause of any of the CTCLs. We have not been able to identify a single cause. There are some risk factors. A 2014 article from the Journal of the National Cancer Institute looked at 324 cases of CTCL and compared it with 17,000 controls.4 They showed some interesting risk factors, such as body mass index (BMI) > 30 and smoking > 40 years. Similar to previous European studies, they showed that occupations like being a farmer, a painter, a woodworker, or a carpenter may carry additional risk.I wonder whether or not veterans were more likely to have some of these risk factors that this epidemiologic study picked up in addition to exposures that they may have encountered during their active-duty service. Interestingly, a decreased risk factor for developing MF was moderate physical activity. Clearly though, there are a large number of patients with CTCL in the veteran population.
I’d like to turn now to some of the challenges with diagnosis. Marianne, could you share some of your experience with early-stage disease and about how long it took them to be diagnosed?
Marianne Tawa, RN, MSN, ANP. Speaking specifically about early-stage disease, patients often share a history of waxing and waning rash that may not be particularly itchy. Confounding the picture, the distribution of early patch or plaque stage CTCL rash frequently occurs in covered areas. Many patients miss out on complete skin examinations by providers, thus early-stage CTCL may not be appreciated in a timely manner.
In certain scenarios, it may take upward of 5 to 7 years before the CTCL diagnosis is rendered. This is not because the patient delayed care. Nor is it because a skin biopsy was not performed. The progression of the disease and meeting the classic features of histology under the microscope can require clinical observation over time and repeated skin biopsies. We recommend patients refrain from topical steroid applications for 2 to 4 weeks prior to skin biopsy if we have a strong suspicion of CTCL. Many patients will report having a chronic eczematous process. Some patients may have a history of parapsoriasis, and they’re on the continuum for CTCL. That’s a common story for CTCL patients.
John Zic. What is the role of a skin biopsy in the diagnosis of CTCL? We see many patients who have had multiple skin biopsies who often wonder whether or not the diagnosis was missed by either the clinician or the pathologist.
Alejandro Ariel Gru. That is a great area of challenge in terms of pathologic diagnosis of early MF. A study led by Julia Scarisbrick, from an international registry data (PROCLIPI) on the early stages of the disease, showed a median delay of diagnosis of early MF of approximately 36 months.5 For all physicians involved in the diagnosis and care of patients with MF, the delay is probably significantly higher than that. We’ve seen patients who have lived without a diagnosis for a period of 10 or sometimes 15 years. That suggests that many cases are behaving in an indolent fashion, and patients are not progressing through the ‘natural’ stages of the disease and remain at the early stage. There also is the potential that other chronic inflammatory conditions, particularly psoriasis or parapsoriasis, can be confused with this entity. The diagnosis of certain types of parapsoriasis, can belong to the same spectrum of MF and can be treated in a similar way than patients with early stage MF are, such as phototherapy or methotrexate.
