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B-CELL LYMPHOMAS
B-CELL LYMPHOMAS
B-cell lymphomas, which are defined as arising primarily in the skin, include:
โข extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma),
โข cutaneous follicle center lymphoma,
โข primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT). The skin may also be involved secondarily in a wide range of mature B-cell lymphomas including, in particular, mantle cell lymphoma (MCL), B-chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), intravascular large B-cell lymphoma (IVLBCL), lymphomatoid granulomatosis, plasmablastic lymphoma (PL), and post-transplant lymphoproliferative disorder (PTLD).
less common.10,11,16,17,22 Lesions may be solitary or localized, affect multiple anatomical sites simultaneously, or rarely be generalized.16,18,19 Exceptionally, a serum monoclonal immunoglobulin is encountered. B-symptoms are absent and serum LDH and beta-2 microglobulin levels are normal.12,18 Anetoderma may rarely be seen in the skin overlying the lesions. Rare cases present following solid organ transplantation.23
Surgery or radiotherapy is used for localized or scattered multifocal lesions, and extensive disease is managed with chemotherapy or electron beam.18,19,24 Most patients achieve complete remission, but cutaneous relapse is common in up to 50% of cases.19
Since B-cell lymphomas presenting in the skin may be either primary or metastatic, staging is essential to distinguish between the two.