Primary cutaneous maringal zone lymphoproliferative disorder

The concept of primary cutaneous marginal zone lymphoma (pcMZL) has been adapted several times in recent years. In 2005, Willemze et al. defined pcMZL as an indolent lymphoma and as part of the group of extranodular B-cell marginal zone lymphomas. In the current version of the WHO classification of lymphoid neoplasms, it was classified as an independent entity in distinction to other marginal zone lymphomas (Alaggio R et al. 2022). In the meantime, pcMZL has been downgraded from a lymphoma to a lymphoproliferative disorder (pcMZLPD) due to its indolent behavior, as the disease-specific survival rate is almost 100% without the need for aggressive therapies. In this respect, various authors refer to this lymphoproliferative entity not as a lymphoma but as a pcMZLPD, a lymphoproliferative disorder (Cankaya R et al. 2025).

1. Alaggio R et al. (2022) The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia 36:1720-1748.
2. Cankaya R et al. (2025) How to treat primary cutaneous B cell lymphoma - Results from a monocentric cohort study on 98 patients. J Dtsch Dermatol Ges 23:822-830.