Presse Med
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PCBCLs are a group of Non-Hodgkin's B-cell lymphomas originating in and usually confined to the skin, representing approximately one fourth of primary cutaneous lymphomas (PCL). Their current classification system has been the result of the joint World Health Organization (WHO) - European Organization for Research and Treatment of Cancer (EORTC) consensus in 2018. ⋯ On the contrary, primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT) is less common, yet more aggressive, with a reported 5-year overall survival of approximatively 50%. In this review, we outline the PCBCLs defining diagnostic criteria, report the features of the less common subtypes and summarize the noteworthy therapeutical options currently available in this field.
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Primary cutaneous lymphomas are a group of T- (CTCL) and B-cell (CBCL) malignancies. These diseases have different clinical presentations and prognosis. Our knowledge on their epidemiology is limited. ⋯ We found that although there are important differences in the epidemiology of cutaneous lymphomas in different countries, the relative frequency of certain, especially rare lymphomas remains stable. Several studies described growing incidences of both CTCL and CBCL. The emergence of new diagnostic criteria, a more precise definition of the entities and new biomarkers enable a better classification of cases.
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PROCLIPI is the PROgnostic Cutaneous Lymphoma International Prognostic Index Study with the main aim to produce a prognostic index in mycosis fungoides (MF) and Sezary syndrome (SS). The study prospectively collects clinical, haematological, pathological, imaging, treatment with responses, quality of life and survival data using careful predefined datasets. Patients are subject to a central review to confirm diagnosis. PROCLIPI opened in 2015 and recruitment has been strong with to date 1916 patients recruited from 52 Centres from 19 countries across 6 continents.
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Primary cutaneous lymphomas are a heterogeneous group of cutaneous T-cell lymphomas (CTCL) and cutaneous B-cell lymphomas (CBCL) that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. In the last decade the 2005 WHO-EORTC consensus classification has served as a golden standard for the diagnosis and classification of these conditions. ⋯ Herein, the characteristic features of the different types of CTCL and CBCL are presented, differences with previous classification schemes discussed and the results of more recent molecular studies with clinical implications for these conditions reviewed. In addition, an update of the frequency and survival of the different types of primary cutaneous lymphomas is provided.