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

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, Bindu, Amel-Kashipaz, R, Marinos, L, Oikonomidi, A, Stratigos, A, Vignon-Pennamen, M-D, Battistella, M, Climent, F, Gonzalez-Barca, E, Georgiou, E, Senetta, R, Zinzani, P, Vakeva, L, Ranki, A, Busschots, A-M, 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-D, Enz, P, Pujol, R, Quint, K, Geskin, L, Hong, E, Evison, Felicity, Vermeer, M, Cerroni, L, Kempf, W, Kim, Y and Willemze, R (2019) The PROCLIPI international registry of early-stage mycosis fungoides identifies substantial diagnostic delay in most patients. The British journal of dermatology, 181 (2). pp. 350-357. ISSN 1365-2133. This article is available to all UHB staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their UHB Athens login IDs.

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Official URL: https://onlinelibrary.wiley.com/doi/full/10.1111/b...

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.

Item Type: Article
Additional Information: This article is available to all UHB staff and students via ASK Discovery tool http://tinyurl.com/z795c8c by using their UHB Athens login IDs.
Subjects: QY Clinical pathology
QZ Pathology. Oncology
WR Skin. Dermatology
Divisions: Ambulatory Care > Dermatology
Clinical Support
Clinical Support > Pathology
Related URLs:
Depositing User: Mrs Noomi Tyholdt-Pidgley
Date Deposited: 08 Jan 2021 15:15
Last Modified: 08 Jan 2021 15:15
URI: http://www.repository.uhblibrary.co.uk/id/eprint/3813

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