Killer Immunoglobulin-Like Receptor Transcripts Expression for the Diagnosis of Epidermotropic Cutaneous T Cell Lymphoma
Condition: Mycosis Fungicides; Sezary Syndrome; Dermatitis; Dermatitis, Exfoliative
MeSH terms: Mycoses; Lymphoma; Lymphoma, T-Cell; Sezary Syndrome; Lymphoma, T-Cell, Cutaneous; Dermatitis; Dermatitis, Exfoliative; Syndrome
Study type: Interventional

Summary: 
      The most frequent cutaneous T-cell lymphomas (CTCL) are mycosis fungoid and Sezary syndrome.
      The diagnosis of these lymphomas is difficult using current methods, especially because
      numerous benign dermatological 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. To address this issue, we will study
      the expression of all known KIR receptor in the skin of patients presenting with a skin
      eruption, which may correspond to either a cutaneous T-cell lymphoma or a benign
      dermatological disease. The final diagnosis will be established by a panel of experts,
      allowing constitution of 2 groups of patients : the cutaneous T-cell lymphoma group, and the
      benign inflammatory disease group. The expression of the different KIRs will be analyzed in
      both group in a blinded fashion, in order to determine whether one or a several KIRs may be
      differentially expressed.
    

Detailed description: 
      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
      dermatoses.

      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.
    

Intervention name(s): Detection of KIR receptor by RT PCR
Intervention type(s): Other
Intervention description(s): Detection on biopsy cutaneous (3mm) and on blood sample of 30 ml
Allocation: Non-Randomized
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Diagnostic
Keywords: Receptors KIR; Polymerase chain reaction; Diagnosis; Lymphoma T-cell cutaneous; Mycosis fungicides; Sezary syndrome; Differential diagnosis from dermatitis