EORTC/USCLC/ISCL consensus recommendations for management and treatment of cutaneous lymphoproliferative disorders

Background In recent classifications several cutaneous lymphomas were reclassified as lymphoproliferative disorder (LPD). These include primary cutaneous CD4-positive small/medium T-cell LPD (PCSM-TCLPD), primary cutaneous acral CD8+ T-cell LPD (acral CD8+ TLPD) and primary cutaneous marginal zone LPD (PCMZLPD; still classified as primary cutaneous marginal zone lymphoma (PCMZL) in the 5th edition of the WHO classification). The results of a survey among 30 cutaneous lymphoma centres on the effects of this new terminology on clinical management revealed considerable heterogeneity and emphasized the need to develop uniform recommendations for management and treatment of these disorders. Objectives To develop consensus recommendations for staging, treatment and follow-up policy in PCSM-TCLPD, acral CD8+ TLPD and PCMZL/LPD. Methods Two surveys with questions regarding staging, treatment and follow-up strategy of cutaneous LPDs were distributed among 30 cutaneous lymphoma expert centres collaborating within the EORTC-CLTG, USCLC and ISCL. Based on the results of these surveys, an extensive literature search, two rounds of feedback and a final consensus meeting consensus recommendations were formulated. Results Important changes compared to current practise and literature include: staging examinations apart from thorough clinical examination of skin and peripheral lymph nodes not required in typical cases of PCSM-TCLPD and acral CD8+ TLPD; low-dose radiotherapy (4-8 Gy) rather than dose ≥20 Gy, for PCSM-TCLPD and acral CD8+ TLPD, and 4 Gy for PCMZL/LPD, with possibility to escalate to a higher dose (20-24 Gy) in case of local failure; increase in the use of intralesional corticosteroids as initial treatment in all three LPDs; limited follow-up period (2 years) in PCSM-TCLPD and acral CD8+ TLPD LPD. Conclusion These EORTC/USCLC/ISCL consensus recommendations reflect the state-of-the-art management and treatment as agreed upon by major cutaneous lymphoma centers. They may contribute to uniform staging, treatment and follow-up policy in patients with cutaneous LPDs.