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BACKGROUND: The PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study is a prospective analysis of an international database. Here we examine front-line treatments and quality of life (QoL) in patients with newly diagnosed mycosis fungoides (MF). OBJECTIVES: To identify (i) differences in first-line approaches according to tumour-nodes-metastasis-blood (TNMB) staging; (ii) parameters related to a first-line systemic approach and (iii) response rates and QoL measures. METHODS: In total, 395 newly diagnosed patients with early-stage MF (stage IA-IIA) were recruited from 41 centres in 17 countries between 1 January 2015 and 31 December 2018 following central clinicopathological review. RESULTS: The most common first-line therapy was skin-directed therapy (SDT) (322 cases, 81·5%), while a smaller percentage (44 cases, 11·1%) received systemic therapy. Expectant observation was used in 7·3%. In univariate analysis, the use of systemic therapy was significantly associated with higher clinical stage (IA, 6%; IB, 14%; IIA, 20%; IA-IB vs. IIA, P < 0·001), presence of plaques (T1a/T2a, 5%; T1b/T2b, 17%; P < 0·001), higher modified Severity Weighted Assessment Tool (> 10, 15%; ≤ 10, 7%; P = 0·01) and folliculotropic MF (FMF) (24% vs. 12%, P = 0·001). Multivariate analysis demonstrated significant associations with the presence of plaques (T1b/T2b vs. T1a/T2a, odds ratio 3·07) and FMF (odds ratio 2·83). The overall response rate (ORR) to first-line SDT was 73%, while the ORR to first-line systemic treatments was lower (57%) (P = 0·027). Health-related QoL improved significantly both in patients with responsive disease and in those with stable disease. CONCLUSIONS: Disease characteristics such as presence of plaques and FMF influence physician treatment choices, and SDT was superior to systemic therapy even in patients with such disease characteristics. Consequently, future treatment guidelines for early-stage MF need to address these issues.

BACKGROUND: Early-stage mycosis fungoides (MF) includes involvement of dermatopathic lymph nodes (LNs) or early lymphomatous LNs. There is a lack of unanimity among current guidelines regarding the indications for initial staging imaging in early-stage presentation of MF in the absence of enlarged palpable LNs. OBJECTIVES: To investigate how often imaging is performed in patients with early-stage presentation of MF, to assess the yield of LN imaging, and to determine what disease characteristics promoted imaging. METHODS: A review of clinicopathologically confirmed newly diagnosed patients with cutaneous patch/plaque (T1/T2) MF from PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) data. RESULTS: PROCLIPI enrolled 375 patients with stage T1/T2 MF: 304 with classical MF and 71 with folliculotropic MF. Imaging was performed in 169 patients (45%): 83 with computed tomography, 18 with positron emission tomography-computed tomography and 68 with ultrasound. Only nine of these (5%) had palpable enlarged (≥ 15 mm) LNs, with an over-representation of plaques, irrespectively of the 10% body surface area cutoff that distinguishes T1 from T2. Folliculotropic MF was not more frequently imaged than classical MF. Radiologically enlarged LNs (≥ 15 mm) were detected in 30 patients (18%); only seven had clinical lymphadenopathy. On multivariate analysis, plaque presentation was the sole parameter significantly associated with radiologically enlarged LNs. Imaging of only clinically enlarged LNs upstaged 4% of patients (seven of 169) to at least IIA, whereas nonselective imaging upstaged another 14% (24 of 169). LN biopsy, performed in eight of 30 patients, identified N3 (extensive lymphomatous involvement) in two and N1 (dermatopathic changes) in six. CONCLUSIONS: Physical examination was a poor determinant of LN enlargement or involvement. Presence of plaques was associated with a significant increase in identification of enlarged or involved LNs in patients w

Zusammenfassung: Primär kutane Lymphome sind die zweithäufigste Gruppe der extranodalen Non-Hodgkin-Lymphome. In den letzten Jahren sind zahlreiche neue Varianten und Entitäten beschrieben worden, wobei diese z.T. noch keinen Eingang in die aktuelle WHO-Klassifikation gefunden haben. Dazu gehören Formen der Mycosis fungoides wie die granulomatöse Form, welche mit einer schlechteren Prognose einhergehen, aber auch die indolenten CD8-positiven Lymphoproliferationen im Kopfbereich und an akralen Lokalisationen. In der Gruppe der CD30-positiven lymphoproliferativen Erkrankungen werden bei der lymphomatoiden Papulose die neuen histologischen TypenD (epidermotrop, CD8+) und TypE (angioinvasiv) besprochen, welche aggressive Lymphome imitieren. Die kutanen peripheren T-Zell-Lymphome sind eine prognostisch heterogene Gruppe. Das kutane CD8+ aggressive epidermotrope zytotoxische Lymphom und das kutane gamma/delta-T-Zell-Lymphom sind ausgesprochen aggressive Erkrankungen, während das kutane CD4+ klein-/mittelgroßzellige T-Zell-Lymphom in seiner solitären und lokalisierten Form eine indolente Lymphomform darstellt, dessen Histogenese, Terminologie und Abgrenzung vom nodulären T-Zell-Pseudolymphom noch nicht geklärt sind. Bei den seltenen B-Zell-Lymphomen, welche sich primär oder sekundär in der Haut manifestieren, wird auf das Epstein-Barr-Virus(EBV)-assoziierte diffuse großzellige Lymphom des älteren Menschen und das EBV-assoziierte mukokutane Ulkus eingegangen. Diese Übersichtsarbeit beschreibt die klinischen, histologischen und immunphänotypischen Merkmale der neuen und seltenen Varianten und Entitäten kutaner Lymphome und zeigt den hohen Stellenwert der klinisch-pathologischen Korrelation in der Diagnostik auf.