Cited by 244Cutaneous lymphoproliferative disorders researchLymphoma Diagnosis and TreatmentNonmelanoma Skin Cancer Studies

The PROCLIPI international registry of early- stage mycosis fungoides identifies substantial diagnostic delay in most patients

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Scarisbrick, J.J., Quaglino, P., Prince, H.M., Papadavid, E., Hodak, E., Bagot, M., Servitje, O., Berti, E. +98 more·2019·British Journal of Dermatology

Abstract

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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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.
Venue
British Journal of Dermatology
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
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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