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Review
. 2023 Apr 27;21(1):283.
doi: 10.1186/s12967-023-04130-6.

Taxonomic reclassification of Kaposi Sarcoma identifies disease entities with distinct immunopathogenesis

M R Openshaw  1   2 , E Gervasi  3 , C A M Fulgenzi  4   5 , D J Pinato  4   6 , A Dalla Pria  7 , M Bower  7
Affiliations
Review

Taxonomic reclassification of Kaposi Sarcoma identifies disease entities with distinct immunopathogenesis

M R Openshaw et al. J Transl Med. .

Abstract

Background: The taxonomy of Kaposi Sarcoma (KS) is based on a classification system focused on the description of clinicopathological features of KS in geographically and clinically diverse populations. The classification includes classic, endemic, epidemic/HIV associated and iatrogenic KS, and KS in men who have sex with men (MSM). We assessed the medical relevance of the current classification of KS and sought clinically useful improvements in KS taxonomy.

Methods: We reviewed the demographic and clinicopathological features of 676 patients with KS, who were referred to the national centre for HIV oncology at Chelsea Westminster hospital between 2000 and 2021.

Results: Demographic differences between the different subtypes of KS exist as tautological findings of the current classification system. However, no definitive differences in clinicopathological, virological or immunological parameters at presentation could be demonstrated between the classic, endemic or MSM KS patients. Reclassifying patients as either immunosuppressed or non-immunosuppressed, showed that the immunosuppressed group had a significantly higher proportion of adverse disease features at presentation including visceral disease and extensive oral involvement, classified together as advanced disease (chi2 P = 0.0012*) and disseminated skin involvement (chi2 P < 0.0001*). Immunosuppressed patients had lower CD4 counts, higher CD8 counts and a trend towards higher HHV8 levels compared to non-immunosuppressed patients, however overall survival and disease specific (KS) survival was similar across groups.

Conclusion: The current system of KS classification does not reflect meaningful differences in clinicopathological presentation or disease pathogenesis. Reclassification of patients based on the presence or absence of immunosuppression is a more clinically meaningful system that may influence therapeutic approaches to KS.

Keywords: Acquired immune deficiency syndrome; Disease classification; Human herpes virus 8; Human immunodeficiency virus; Kaposi Sarcoma.

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Conflict of interest statement

David J Pinato received lecture fees from ViiV Healthcare, Bayer Healthcare, BMS, Roche, EISAI, Falk Foundation, travel expenses from BMS and Bayer Healthcare; consulting fees for Mina Therapeutics, EISAI, Roche, DaVolterra and Astra Zeneca; research funding (to institution) from MSD and BMS. Mark R Openshaw received travel expenses from Servier. No other conflicts of interest.

Figures

Fig. 1
Fig. 1
Consort diagram of patients diagnosed at Chelsea and Westminster Hospital, according to current classification system. MSM = men who have sex with men
Fig. 2
Fig. 2
Blood parameters including HHV8 viral load and CD4 counts. A Box Plot of mean Log Blood HHV8 levels. B Box Plot of CD4 counts (cells/µl)
Fig. 3
Fig. 3
Overall survival of patients with KS. A Overall survival in current subtypes of KS. B Overall survival in immunocompromised versus non-immunocompromised patients
Fig. 4
Fig. 4
Disease Specific Survival of patients with KS. A Disease specific survival in subtypes of KS. B Disease specific survival in immunocompromised versus non-immunocompromised patients
Fig. 5
Fig. 5
Comparison of demographic and clinicopathological characteristics, and blood parameters at presentation of KS when classified as immunosuppressed versus non-immunosuppressed. A Presenting Skin sites of KS lesions B Sites of advanced KS involvement at diagnosis. C Log blood HHV8 viral load and CD4 count at diagnosis
See this image and copyright information in PMC

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