Analysis of Killer Immunoglobulin-like Receptor Transcripts Expression for the Diagnosis of Epidermotropic Cutaneous T-cell Lymphomas (Mycosis Fungoid and Sézary Syndrome) in Patients With Erythroderma or Erythematous Patches/Plaques.
Background : The most frequent cutaneous T-cell lymphomas (CTCL) are mycosis fungoid and
Sezary syndrome. Both are due to the proliferation of a CD4+ T-cell clone in the skin,
associated with a blood involvement in Sezary syndrome. Mycosis fungoid clinically presents
as a patches or plaques dermatitis and Sezary syndrome as an exfoliative dermatitis. The
diagnosis of these lymphomas is difficult using current methods, especially because numerous
benign dermatological inflammatory conditions can mimick CTCL both clinically and under
microscopic examination. Recently, the KIR receptor CD158k has been shown to be a marker for
Sezary syndrome in both the blood and skin. We hypothesize that other receptors from the
same family may help fro the diagnosis of these lymphomas.
Aim of the study : to determine if one or a panel of KIR(s) receptor(s) may help for the
differential diagnosis between cutaneous T-cell lymphoma (CTCL) and benign inflammatory
Subjects selection : all patients presenting to an investigator, member of the GFELC experts
group ("French Group Study Cutaneous Lymphoma"), with either an exfoliative or patch/plaque
dermatitis with a clinical suspicion of CTCL will be enrolled.
Number of subjects : A total of 550 patients could be recruited by the GFELC, including 180
CTCL (60 Sezary syndrome and 120 mycosis fungoid) and 370 inflammatory diseases (240 patch
dermatitis and 130 exfoliative dermatitis).
Inclusion period : patients will be included during a 2 years period and will be followed
during 6 months. Total study length will be 30 months.
Interventions : 1) 3 mm punch skin biopsy for all patients 2) 10 ml blood sample for
patients with exfoliative dermatitis Methods : Following initial and 6 month follow-up
evaluations, patients will be classified in one of the following groups : the cutaneous
T-cell lymphoma group, and the benign inflammatory disease group. The expression of all
known KIRs receptors (KIR2DL1 (CD158a), KIR2DL2 (CD158b1), KIR2DL3 (CD158b2), KIR2DL4
(CD158d), KIR2DL5 (CD158f), KIR3DL1 (CD158e1), KIR3DL2 (CD158k), KIR2DS1 (CD158h), KIR2DS2
(CD158j), KIR2DS4 (CD158i), KIR2DS5 (CD158g), KIR3DS1 (CD158e2)) will be evaluated using
reverse transcription and quantitative polymerase chain reaction in all skin and blood
samples, in a blinded fashion. For blood samples, the analyses will be performed on CD4+
T-cell sorted using magnetic beads.
Outcome measures : The main outcome measure will be the differential expression of one or a
panel of KIR(s) receptor(s) between CTCL and benign inflammatory diseases. Secondary outcome
measure will be a differential quantitative expression of one or a panel of KIR(s)
receptor(s) between the two groups.
Allocation: Non-Randomized, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Diagnostic
Differential expression of one or a panel of KIRs transcript(s) between epidermotropic cutaneous lymphoma and inflammatory diseases
at the inclusion
Nicolas Ortonne, MD
Assistance Publique - Hôpitaux de Paris
France: Ministry of Health