Updated review on prognostic factors in mycosis fungoides and new skin lymphoma trials

Banu Farabi; Lucia Seminario-Vidal; Marielle Jamgochian; Bengu Nisa Akay; Mehmet Fatih Atak; Babar K Rao; Priyanka Karagaiah; Stephan Grabbe; Mohamad Goldust

doi:10.1111/jocd.14528

Updated review on prognostic factors in mycosis fungoides and new skin lymphoma trials

J Cosmet Dermatol. 2022 Jul;21(7):2742-2748. doi: 10.1111/jocd.14528. Epub 2021 Oct 23.

Authors

Banu Farabi¹, Lucia Seminario-Vidal², Marielle Jamgochian³, Bengu Nisa Akay⁴, Mehmet Fatih Atak⁴, Babar K Rao⁵, Priyanka Karagaiah⁶, Stephan Grabbe⁷, Mohamad Goldust⁸

Affiliations

¹ Department of Internal Medicine, Saint Peter's University Hospital, New Brunswick, New Jersey, USA.
² Department of Dermatology, Department of Cutaneous Oncology, Moffitt Cancer Center and Research Institute, University of South Florida Health Morsani College of Medicine, Tampa, Florida, USA.
³ Department of Dermatology, Robert Wood Johnson Medical Center, Rutgers University, New Brunswick, New Jersey, USA.
⁴ Dermatology Department, Ankara University, Ankara, Turkey.
⁵ Dermatology Department, Weil Cornell Medicine, NY, Somerset, New Jersey, USA.
⁶ Department of dermatology, Bangalore Medical College and Research Institute, Bangalore, India.
⁷ Department of Dermatology, University Medical Center of the Johannes Gutenberg University, Mainz, Germany.
⁸ Department of Dermatology, University Medical Center Mainz, Mainz, Germany.

PMID: 34687485
DOI: 10.1111/jocd.14528

Abstract

Background: Ten-year survival rates in mycosis fungoides (MF) broadly varies, however, there is no standardized prognostic index available. This is presumably due to low prevalence, heterogeneity, and diagnostic challenges in MF. Recent studies have focused on identifying objective prognostic indices by using different parameters for survival determinants. The Cutaneous Lymphoma International Prognostic Index (CLIPI) and the Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) represent prototypical studies that identify prognostic factors, seeking to improve management and outcomes in early-stage MF. Detecting these factors and stratifying MF patients according to their disease progression risk may help to manage these patients more efficiently.

Aims: Review the current literature to determine the risk factors determining prognosis in MF.

Methodology: A Comprehensive literature search was performed using electronic online databases "PubMed" and "Google Scholar" using key words 'prognostic factor', 'prognostic indicator', 'mycosis fungoides', 'Sezary syndrome', 'Skin Lymphoma', 'Cutaneous Lymphoma'. Articles published in English language were considered for the review.

Results: The strongest prognostic factor in MF patients is the stage of the disease. T stage and the presence of extracutaneous disease are the most important factors for survival. Other factors that are associated with worse prognosis are male gender, age >60, presence of plaques, folliculotropism, eosinophilia and lymph node stage above N1/Nx. Elevated LDH was associated with later tumor stages and large cell phenotype at diagnosis had a better prognosis. KIR3DL2 was associated with malignant transformation.

Conclusion: The PROCLIPI study has assessed risk factors collected in MF patients from different countries and across different ethnicities following a rigorous clinicopathologic process. The findings presented here illustrated that disease prognosis in early stages depends on many contributing factors. Detection and stratification of such factors may allow a personalized approach to management of these patients.

Keywords: cutaneous lymphoma international prognostic index; mycosis fungoides; prospective cutaneous lymphoma international prognostic index; skin lymphoma.

Publication types

Review

MeSH terms

Female
Humans
Male
Mycosis Fungoides* / diagnosis
Mycosis Fungoides* / therapy
Neoplasm Staging
Prognosis
Prospective Studies
Skin Neoplasms* / diagnosis
Skin Neoplasms* / therapy