The PROCLIPI international registry of early- stage mycosis fungoides identifies substantial diagnostic delay in most patients
Open accessAbstract
BACKGROUND: Survival in mycosis fungoides (MF) is varied and may be poor. The PROCLIPI (PROspective Cutaneous Lymphoma International Prognostic Index) study is a web-based data collection system for early-stage MF with legal data-sharing agreements permitting international collaboration in a rare cancer with complex pathology. Clinicopathological data must be 100% complete and in-built intelligence in the database system ensures accurate staging. OBJECTIVES: To develop a prognostic index for MF. METHODS: Predefined datasets for clinical, haematological, radiological, immunohistochemical, genotypic, treatment and quality of life are collected at first diagnosis of MF and annually to test against survival. Biobanked tissue samples are recorded within a Federated Biobank for translational studies. RESULTS: In total, 430 patients were enrolled from 29 centres in 15 countries spanning five continents. Altogether, 348 were confirmed as having early-stage MF at central review. The majority had classical MF (81·6%) with a CD4 phenotype (88·2%). Folliculotropic MF was diagnosed in 17·8%. Most presented with stage I (IA: 49·4%; IB: 42·8%), but 7·8% presented with enlarged lymph nodes (stage IIA). A diagnostic delay between first symptom development and initial diagnosis was frequent [85·6%; median delay 36 months (interquartile range 12-90)]. This highlights the difficulties in accurate diagnosis, which includes lack of a singular diagnostic test for MF. CONCLUSIONS: This confirmed early-stage MF cohort is being followed-up to identify prognostic factors, which may allow better management and improve survival by identifying patients at risk of disease progression. This study design is a useful model for collaboration in other rare diseases, especially where pathological diagnosis can be complex.
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Cutaneous TCR clonality is confirmed in only 64% of early-stage patients.
“Cutaneous TCR clonality is confirmed in only 64% of earlystage patients”
From Histological triage of early-stage mycosis fungoides using a weakly supervised deep learning-based model: a multicentre, external validation, and clinical utility study (2026) · cites this paper as [2]
The Results (Accepted Article) report cutaneous T-cell receptor clonality in 132 of 205 tested patients (64.4%) with early-stage mycosis fungoides. Because not all patients were tested, the paper does not establish that clonality was confirmed in 64% of all early-stage patients. (LLM, conf=0.86)
Experimental — accuracy not yet measuredEarly-stage MF diagnosis results in a median diagnostic delay of 36 months from symptom onset.
“This”
Detection of aberrant pan-T-cell marker loss carries a sensitivity as low as 10%.
“the detection of aberrant pan-T-cell marker loss carries a sensitivity as low as 10%”
From Histological triage of early-stage mycosis fungoides using a weakly supervised deep learning-based model: a multicentre, external validation, and clinical utility study (2026) · cites this paper as [2]
The Skin Histology and Clonality section reports CD4 and CD8 staining patterns, while the Accepted Article discussion addresses the sensitivity of T-cell clonality testing—not pan-T-cell marker loss. Searches for pan-T-cell marker loss, CD7 loss, and a 10% sensitivity found no passage in our copy supporting that figure; the retrieved passages have no page numbers. (LLM, conf=0.75)
Experimental — accuracy not yet measured
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Primary cutaneous T-cell lymphoma: a review of the most common entities with focus on recent updates
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Diagnosis and Management of Cutaneous Lymphomas Including Cutaneous T-cell Lymphoma
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Chlormethine Gel is Efficient and Safe in Mycosis Fungoides Skin Lesions
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Self-citationMycosis fungoides and Sézary syndrome – Review and outlook
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Typical and Atypical Imaging Features of Malignant Lymphoma in the Abdomen and Mimicking Diseases
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Self-citationDiagnostic Two-Gene Classifier in Early-Stage Mycosis Fungoides: A Retrospective Multicenter Study
Nielsen, P. R., Eriksen, J. O., Lindahl, L. M., Wehkamp, U., Bzorek, M., Andersen, G., Woetmann, A., Iversen, L., Ødum, N., Litman, T., et al. (2020) · Journal of Investigative Dermatology · doi:10.1016/j.jid.2020.04.026 · Cited by 10 · see citing works
Self-citationUnderstanding racial disparities in mycosis fungoides through international collaborative studies
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Self-citationRole of Neutrophil-to-Lymphocyte Ratio (NLR) in Patients with Mycosis Fungoides
Raimondo, C. D., Lombardo, P., Tesei, C., Esposito, F., Meconi, F., Secchi, R., Lozzi, F., Monopoli, A., Narducci, M. G., Scala, E., et al. (2023) · Diagnostics · doi:10.3390/diagnostics13111979 · Cited by 8 · see citing works
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Self-citationAdvances in the pharmacological management of cutaneous T-cell lymphoma
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Self-citationMode of action, indications and recommendations on extracorporeal photopheresis (ECP)
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Self-citationPrognosis of increasing percentages of lesional body surface area in early stage mycosis fungoides
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Self-citationMolecular pathogenesis of cutaneous lymphoma–Future directions
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Self-citationMycosis Fungoides and Associated Malignancies in a Dutch Nationwide Retrospective Cohort Study
Ottevanger, R., Vermaas, E., Willemze, R., Schrader, A. M. R., Jansen, P. M., Goeman, J. J., Putter, H., Vermeer, M. H., & Quint, K. D. (2024) · Acta Dermato Venereologica · doi:10.2340/actadv.v104.40065 · Cited by 6 · see citing works
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Self-citationClinical characteristics of primary cutaneous lymphoma: analysis from two centres in China
Shi, H., Liu, Y. ‐., Jiang, Y., Chen, S., Xu, X., Lu, X., Zhang, W., Zeng, X. ‐., Zhou, G., Sun, J., et al. (2019) · British Journal of Dermatology · doi:10.1111/bjd.18266 · Cited by 6 · see citing works
- Authors
- Scarisbrick, J. J., Quaglino, P., Prince, H. M., Papadavid, E., Hodak, E., Bagot, M., Servitje, O., Berti, E., Ortiz‐Romero, P., Stadler, R., Patsatsi, A., Knobler, R., Guenova, E., Child, F., Whittaker, S., Nikolaou, V., Tomasini, C., Amitay, I., Prag Naveh, H., Ram‐Wolff, C., Battistella, M., Alberti‐Violetti, S., Stranzenbach, R., Gargallo, V., Muniesa, C., Koletsa, T., Jonak, C., Porkert, S., Mitteldorf, C., Estrach, T., Combalia, A., Marschalko, M., Csomor, J., Szepesi, A., Cozzio, A., Dummer, R., Pimpinelli, N., Grandi, V., Beylot‐Barry, M., Pham‐Ledard, A., Wobser, M., Geissinger, E., Wehkamp, U., Weichenthal, M., Cowan, R., Parry, E., Harris, J., Wachsmuth, R., Turner, D., Bates, A., Healy, E., Trautinger, F., Latzka, J., Yoo, J., Vydianath, B., Amel‐Kashipaz, R., Marinos, L., Oikonomidi, A., Stratigos, A., Vignon‐Pennamen, M. ‐., Battistella, M., Climent, F., Gonzalez‐Barca, E., Georgiou, E., Senetta, R., Zinzani, P., Vakeva, L., Ranki, A., Busschots, A. ‐., Hauben, E., Bervoets, A., Woei‐A‐Jin, F. J. S. H., Matin, R., Collins, G., Weatherhead, S., Frew, J., Bayne, M., Dunnill, G., McKay, P., Arumainathan, A., Azurdia, R., Benstead, K., Twigger, R., Rieger, K., Brown, R., Sanches, J. A., Miyashiro, D., Akilov, O., McCann, S., Sahi, H., Damasco, F. M., Querfeld, C., Folkes, A., Bur, C., Klemke, C. ‐., Enz, P., Pujol, R., Quint, K., Geskin, L., Hong, E., Evison, F., Vermeer, M., Cerroni, L., Kempf, W., Kim, Y., & Willemze, R.
- Affiliations
- European Organisation for Research and Treatment of Cancer (NL)
- Member of the Cutaneous Lymphoma International Consortium (CLIC)
- Member of the European Organisation of Research and Treatment of Cancer (EORTC) Cutaneous Lymphoma Task Force
- Member of the UK Cutaneous Lymphoma Group
- European Co‐ordinating PROCLIPI Centre for PROCLIPI University Hospitals Birmingham Birmingham U.K.
- Corresponding author
- Scarisbrick, J.J.
- ORCID
- Scarisbrick, J.J.
- Quaglino, P.
- Prince, H.M.
- Papadavid, E.
- Hodak, E.
- Bagot, M.
- Servitje, O.
- Berti, E.
- Stadler, R.
- Patsatsi, A.
- Knobler, R.
- Guenova, E.
- Whittaker, S.
- Nikolaou, V.
- Tomasini, C.
- Ram‐Wolff, C.
- Stranzenbach, R.
- Jonak, C.
- Porkert, S.
- Mitteldorf, C.
- Estrach, T.
- Cozzio, A.
- Dummer, R.
- Pimpinelli, N.
- Grandi, V.
- Pham‐Ledard, A.
- Wobser, M.
- Geissinger, E.
- Wehkamp, U.
- Weichenthal, M.
- Cowan, R.
- Bates, A.
- Healy, E.
- Trautinger, F.
- Yoo, J.
- Marinos, L.
- Stratigos, A.
- Vignon‐Pennamen, M.‐D.
- Climent, F.
- Georgiou, E.
- Senetta, R.
- Zinzani, P.
- Ranki, A.
- Busschots, A.‐M.
- Hauben, E.
- Bervoets, A.
- Matin, R.
- Collins, G.
- Weatherhead, S.
- McKay, P.
- Benstead, K.
- Rieger, K.
- Brown, R.
- Sanches, J.A.
- Miyashiro, D.
- Akilov, O.
- Sahi, H.
- Querfeld, C.
- Enz, P.
- Pujol, R.
- Quint, K.
- Geskin, L.
- Evison, F.
- Vermeer, M.
- Cerroni, L.
- Kempf, W.
- Kim, Y.
- Willemze, R.
- Venue
- British Journal of Dermatology
- Publisher
- Oxford University Press (OUP)
- Published
- 2018-11-25
- Volume
- 181
- Issue
- 2
- Pages
- 350-357
- ISSN
- 0007-0963 (ISSN-L)
- 1365-2133
- OpenAlex
- W2893877319
- Cited by
- 244
- References
- 37
- Citations by year
- 2026: 30 · 2025: 27 · 2024: 38 · 2023: 52 · 2022: 21 · 2021: 31 · 2020: 25 · 2019: 17 · 2018: 1
- FWCI
- 20.01
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- British Journal of Dermatology (publishedVersion)
- PubMed (publishedVersion)
- ePrints Soton (University of Southampton) (acceptedVersion)
- Archivio istituzionale della ricerca (Alma Mater Studiorum Università di Bologna) (submittedVersion, cc-by-nc-nd)
- IRIS (submittedVersion)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion)
- Minerva Access (University of Melbourne) (submittedVersion)
- Lirias (KU Leuven) (submittedVersion)
- SERVAL (Université de Lausanne) (submittedVersion)
- Zurich Open Repository and Archive (University of Zurich) (submittedVersion)
- Funders
- Cancer Research UK (50763/A18021)
- Topics
- Health Sciences › Medicine › Dermatology › Cutaneous lymphoproliferative disorders research
- Health Sciences › Medicine › Pathology and Forensic Medicine › Lymphoma Diagnosis and Treatment
- Health Sciences › Medicine › Epidemiology › Nonmelanoma Skin Cancer Studies
- Keywords
- mycosis fungoides, cutaneous T-cell lymphoma, early-stage disease, delayed diagnosis, prognostic score, folliculotropic mycosis fungoides, disease staging, survival, international cooperation
- MeSH terms
- Adult, Age Factors, Aged, Female, Follow-Up Studies, Humans, International Cooperation, Male, Middle Aged, Mycosis Fungoides, Neoplasm Staging, Prognosis, Prospective Studies, Registries, Risk Factors, Skin, Skin Neoplasms, Disease Progression, Delayed Diagnosis, Datasets as Topic
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