Blood Markers for Differentiating Mycosis Fungoides and Sézary Syndrome

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• This study included 156 Sézary syndrome (SS), 36 erythrodermic mycosis fungoides (MF), 185 early‐stage MF, 30 erythrodermic benign inflammatory dermatoses (eBID), 27 non–erythrodermic BID, and 13 other cutaneous T-cell lymphoma patients who underwent testing with RT-qPCR for biomarkers T-plastin, Twist, KIR3DL2, NKp46, and Tox. The combination of T-plastin and Twist were able to differentiate erythrodermic SS from erythrodermic presentations of MF or BID suggesting usefulness in differentiating these diseases. Among 107 patients followed further for prognostic data, KIR3DL2 was the only biomarker that demonstrated prognostic utility in predicting time to relapse, with a positive value associated with an earlier relapse.

• T‐plastin, Twist, and KIR3DL2 had the best validity and may be useful serum diagnostic markers for SS. Importantly, the biomarkers did not differentiate early MF from BID, and so could not be used for diagnosis of MF.

– Caroline K. Crabtree, MD

Abstract 

BACKGROUND

Clinical and histological diagnosis of Sézary syndrome (SS) and mycosis fungoides (MF) is challenging in clinical routine.

OBJECTIVES

We investigated five blood markers previously described for SS (T-plastin, Twist, KIR3DL2, NKp46 and Tox) in a prospective validation cohort of patients.

METHODS

We included 447 patients in this study and 107 patients were followed up for prognosis. The markers were analysed by reverse transcriptase quantitative real-time polymerase chain reaction (RT-qPCR) on peripheral blood leucocytes and CD4+ T cells in a cohort of consecutive patients with early MF, erythrodermic MF and SS and compared with patients presenting with benign inflammatory dermatoses (BID) and erythrodermic BID. The markers were assessed in parallel to gold standard values such as CD4/CD8 ratio, loss of CD7 and CD26 membrane expression and CD4 absolute values. Sensitivity and specificity were analysed by receiver operator characteristic curves. The prognostic value of selected markers was analysed on a subset of patients. This study was conducted in one centre.

RESULTS

We defined cut-off values for each marker. T-plastin, Twist and KIR3DL2 had the best validity. SS may be overrepresented. The combination of T-plastin and Twist was able to differentiate between erythrodermic MF or BID and SS. The additional analysis of KIR3DL2 may be useful to predict the prognosis.

CONCLUSIONS

We propose T-plastin, Twist and KIR3DL2 measured by RT-qPCR as new diagnostic markers for Sézary syndrome.

The British Journal of Dermatology

The Value of Five Blood Markers in Differentiating Mycosis Fungoides and Sézary Syndrome: A Validation Cohort

Br J Dermatol 2020 Dec 14;[EPub Ahead of Print], G Dobos, C De Cevins, S Ly Ka So, F Jean-Louis, S Mathieu, C Ram-Wolff, M Resche-Rigon, A Bensussan, M Bagot, L Michel 

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Benjamin Hidalgo-Matlock
Skin Care Physicians of Costa Rica

Clinica Victoria en San Pedro: 4000-1054
Momentum Escazu: 2101-9574

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