Willemze Rein, Assaf Chalid, Bagot Martine, Beylot-Barry Marie, Berti Emilio, Busschots Anne Marie, Cerroni Lorenzo, Climent Fina, Diercks Gilles, Geskin Larisa, Gniadecki Robert, Gru Alejandro A, Guenova Emmanuella, Guitart Joan, Jansen Patty, Kempf Werner, Kim Ellen, Kim Youn H, Koldijk Marjolein, Marschalkó Martá, Mitteldorf Christina, Molgó Montserrat, Muniesa Cristina, Neelis Karen J, Olsen Elise, Ortiz-Romero Pablo L, Papadavid Evangelia, Pimpinelli Nicola, Pulitzer Melissa, Quaglino Pietro, Querfeld Christiane, Quint Koen, Scarisbrick Julia J, Schrader Anne M R, Stadler Rudolf, Vermeer Maarten, Wehkamp Ulrike, Whittaker Sean, Wobser Marion
Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
Department of Dermatology and Venerology, Helios Klinikum Krefeld, Krefeld, Germany.
Br J Dermatol. 2025 Aug 6. doi: 10.1093/bjd/ljaf312.
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.
To develop consensus recommendations for staging, treatment and follow-up policy in PCSM-TCLPD, acral CD8+ TLPD and PCMZL/LPD.
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.
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.
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.
在最近的分类中，几种皮肤淋巴瘤被重新归类为淋巴增殖性疾病（LPD）。这些包括原发性皮肤CD4阳性小/中T细胞LPD（PCSM-TCLPD）、原发性皮肤肢端CD8 + T细胞LPD（肢端CD8 + TLPD）和原发性皮肤边缘区LPD（PCMZLPD；在世界卫生组织分类第5版中仍被归类为原发性皮肤边缘区淋巴瘤（PCMZL））。一项针对30个皮肤淋巴瘤中心的调查结果显示，这种新术语对临床管理的影响存在很大异质性，并强调需要为这些疾病的管理和治疗制定统一的建议。
为PCSM-TCLPD、肢端CD8 + TLPD和PCMZL/LPD的分期、治疗和随访策略制定共识性建议。
向在EORTC-CLTG、USCLC和ISCL内合作的30个皮肤淋巴瘤专家中心分发了两份关于皮肤LPD分期、治疗和随访策略的调查问卷。基于这些调查结果、广泛的文献检索、两轮反馈以及一次最终共识会议，制定了共识性建议。
与当前实践和文献相比的重要变化包括：在典型的PCSM-TCLPD和肢端CD8 + TLPD病例中，除了对皮肤和外周淋巴结进行全面临床检查外，不需要进行分期检查；对于PCSM-TCLPD和肢端CD8 + TLPD，采用低剂量放疗（4 - 8 Gy）而非剂量≥20 Gy，对于PCMZL/LPD采用4 Gy，若局部失败可将剂量增加至更高剂量（20 - 24 Gy）；在所有三种LPD中，增加病灶内注射皮质类固醇作为初始治疗的使用；PCSM-TCLPD和肢端CD8 + TLPD LPD的随访期有限（2年）。
这些EORTC/USCLC/ISCL共识性建议反映了主要皮肤淋巴瘤中心商定的最新管理和治疗方法。它们可能有助于皮肤LPD患者的统一分期、治疗和随访策略。
