The University of Southampton
University of Southampton Institutional Repository
Warning ePrints Soton is experiencing an issue with some file downloads not being available. We are working hard to fix this. Please bear with us.

The PROCLIPI international registry of early stage Mycosis Fungoides identifies substantial diagnostic delay in most patients

The PROCLIPI international registry of early stage Mycosis Fungoides identifies substantial diagnostic delay in most patients
The PROCLIPI international registry of early stage Mycosis Fungoides identifies substantial diagnostic delay in most patients
Survival in mycosis fungoides (MF) is varied and may be poor. 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. Pre-defined 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 with the aim of developing a prognostic index. Biobanked tissue samples are recorded within a Federated Biobank for translational studies. 430 patients were enrolled from 29 Centres in 15 countries spanning 5 continents. 348 were confirmed as 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%), with a median delay of 36 months (interquartile range=12-90 months). This highlights the difficulties in accurate diagnosis, which is contributed by lack of a singular diagnostic test for MF. 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.
0007-0963
Healy, Eugene
400fc04d-f81a-474a-ae25-7ff894be0ebd
Scarisbrick, J.J.
08661d5d-bf5d-4c6a-a683-ddba02ebc92b
Quaglino, P.
071dbf2e-8f60-4990-abd8-06f8e1f83734
Prince, H.M.
61992138-c79d-46a0-8123-fd5db1801c78
PROCLIPI
Healy, Eugene
400fc04d-f81a-474a-ae25-7ff894be0ebd
Scarisbrick, J.J.
08661d5d-bf5d-4c6a-a683-ddba02ebc92b
Quaglino, P.
071dbf2e-8f60-4990-abd8-06f8e1f83734
Prince, H.M.
61992138-c79d-46a0-8123-fd5db1801c78

Scarisbrick, J.J., Quaglino, P. and Prince, H.M. , PROCLIPI (2018) The PROCLIPI international registry of early stage Mycosis Fungoides identifies substantial diagnostic delay in most patients. British Journal of Dermatology. (doi:10.1111/bjd.17258).

Record type: Article

Abstract

Survival in mycosis fungoides (MF) is varied and may be poor. 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. Pre-defined 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 with the aim of developing a prognostic index. Biobanked tissue samples are recorded within a Federated Biobank for translational studies. 430 patients were enrolled from 29 Centres in 15 countries spanning 5 continents. 348 were confirmed as 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%), with a median delay of 36 months (interquartile range=12-90 months). This highlights the difficulties in accurate diagnosis, which is contributed by lack of a singular diagnostic test for MF. 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.

Text
Scarisbrick_et_al-2018-British_Journal_of_Dermatology - Accepted Manuscript
Download (2MB)

More information

e-pub ahead of print date: 29 September 2018

Identifiers

Local EPrints ID: 425952
URI: http://eprints.soton.ac.uk/id/eprint/425952
ISSN: 0007-0963
PURE UUID: be4800ad-93f2-4e2a-9911-d5b6870d5351

Catalogue record

Date deposited: 07 Nov 2018 17:30
Last modified: 26 Nov 2021 06:10

Export record

Altmetrics

Contributors

Author: Eugene Healy
Author: J.J. Scarisbrick
Author: P. Quaglino
Author: H.M. Prince
Corporate Author: PROCLIPI

Download statistics

Downloads from ePrints over the past year. Other digital versions may also be available to download e.g. from the publisher's website.

View more statistics

Atom RSS 1.0 RSS 2.0

Contact ePrints Soton: eprints@soton.ac.uk

ePrints Soton supports OAI 2.0 with a base URL of http://eprints.soton.ac.uk/cgi/oai2

This repository has been built using EPrints software, developed at the University of Southampton, but available to everyone to use.

We use cookies to ensure that we give you the best experience on our website. If you continue without changing your settings, we will assume that you are happy to receive cookies on the University of Southampton website.

×