The Cutaneous B-cell Lymphomas

Primary cutaneous lymphomas (PCLs) are non-Hodgkin lymphomas that are present only in the skin at the time of diagnosis, and without evidence of extracutaneous manifestations. Among extranodal non-Hodgkin lymphomas, PCLs are the second most frequently observed type after gastrointestinal lymphomas. PCLs occur at an incidence of 1 per 100,000 persons annually, and about 25% to 30% of all are Cutaneous B-cell lymphomas (CBCLs). CBCLs can be further classified according to clinical behavior as either aggressive or indolent. Indolent CBCLs include primary cutaneous marginal zone lymphoma (PCMZL) and primary cutaneous follicle center lymphoma (PCFCL), whereas the aggressive CBCLs are diffuse large B-cell lymphoma leg type (DLBCL LT) and intravascular large B-cell lymphoma (IVLBCL). The diagnostic process and selection of therapy are based on histological and immunohistochemical classification, as well as the exclusion of a systemic involvement - which differentiate nodal from systemic lymphomas.

The incidence of CBCLs has risen in recent years, and although classical therapies are most effective in indolent lymphomas, DLBCL LT mortality is still high and the addition of targeted therapies has become an indispensable part of treatment. Furthermore, the pathogenesis and biology of CBCL are not yet fully understood, and there is a need for comprehensive research, especially in the field of DLBCL-LT. The aim of this Research Topic is to describe the clinical features, differential diagnoses, histopathologic features, and new treatment options for each of the four types of CBCL.
 

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