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Primary cutaneous lymphoma
Disease definition
Cutaneous lymphoma is a heterogeneous entity with respect to its clinical and pathological features, evolutive profile, prognosis, molecular aetiology and response to therapy. These specifications have been taken into account in recent classifications, which have placed particular importance on the prognostic implications of these different entities.
ORPHA:542
Classification level: Group of disorders- Synonym(s): -
- Prevalence: 1-9 / 100 000
- Inheritance: -
- Age of onset: -
- ICD-10: -
- OMIM: -
- UMLS: C1302772
- MeSH: -
- GARD: -
- MedDRA: 10051708
Summary
Clinical description
Cutaneous T-cell lymphomas can be subdivided into forms with a good prognosis (Mycosis fungoides and CD30+ cutaneous T-cell lymphoma), forms with a poor prognosis (Sezary syndrome and peripheral T-cell lymphoma) and forms with a variable prognosis (pleomorphic small/medium-sized T-cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, cutaneous T/NK cell lymphomas (referred to as nasal-type lesions), epidermotropic CD8+ T-cell lymphomas and the TCRgamma-delta phenotype). The group of cutaneous B-cell lymphomas includes marginal zone B-cell lymphomas and primary cutaneous follicular lymphomas (both of which have a good prognosis), cutaneous large B-cell lymphomas of the leg (with a variable prognosis), and blastic NK lymphomas (associated with a poor prognosis).
Management and treatment
While treatment is effective and the prognosis is favourable for low grade forms, recent therapeutic innovations relying on the use of biological tools are likely to improve the prognosis for the most severe forms, such as those already existing in the form of anti-CD20 monoclonal antibodies (rituximab) for disseminated cutaneous B-cell lymphomas, and anti-CD52 monoclonal antibody (alemtuzumab) for refractory forms of cutaneous T-cell lymphoma. Deciphering the key molecular mechanisms underlying these malignancies should create new therapeutic perspectives in the close future.
A summary on this disease is available in Deutsch (2007) Español (2007) Français (2007) Italiano (2007) Nederlands (2007)
Detailed information
Guidelines
- Clinical practice guidelines
- Deutsch (2014) - AWMF
Disease review articles
- Review article
- Français (2007, pdf) - Rev Praticien


Additional information