Cutaneous Lymphoma International Consortium Study of Outcome in Advanced Stages of Mycosis Fungoides and Sézary Syndrome: Effect of Specific Prognostic Markers on Survival and Development of a Prognostic Model

Abstract

Purpose Advanced-stage mycosis fungoides (MF; stage IIB to IV) and Sézary syndrome (SS) are aggressive lymphomas with a median survival of 1 to 5 years. Clinical management is stage based; however, there is wide range of outcome within stages. Published prognostic studies in MF/SS have been single-center trials. Because of the rarity of MF/SS, only a large collaboration would power a study to identify independent prognostic markers. Patients and Methods Literature review identified the following 10 candidate markers: stage, age, sex, cutaneous histologic features of folliculotropism, CD30 positivity, proliferation index, large-cell transformation, WBC/lymphocyte count, serum lactate dehydrogenase, and identical T-cell clone in blood and skin. Data were collected at specialist centers on patients diagnosed with advanced-stage MF/SS from 2007. Each parameter recorded at diagnosis was tested against overall survival (OS). Results Staging data on 1,275 patients with advanced MF/SS from 29 international sites were included for survival analysis. The median OS was 63 months, with 2- and 5-year survival rates of 77% and 52%, respectively. The median OS for patients with stage IIB disease was 68 months, but patients diagnosed with stage III disease had slightly improved survival compared with patients with stage IIB, although patients diagnosed with stage IV disease had significantly worse survival (48 months for stage IVA and 33 months for stage IVB). Of the 10 variables tested, four (stage IV, age > 60 years, large-cell transformation, and increased lactate dehydrogenase) were independent prognostic markers for a worse survival. Combining these four factors in a prognostic index model identified the following three risk groups across stages with significantly different 5-year survival rates: low risk (68%), intermediate risk (44%), and high risk (28%). Conclusion To our knowledge, this study includes the largest cohort of patients with advanced-stage MF/SS and identifies markers with independent prognostic value, which, used together in a prognostic index, may be useful to stratify advanced-stage patients.

Innovative Treatment Concepts for Cutaneous T-Cell Lymphoma Based on Microenvironment Modulation

“…Performing univariate analysis of 10 variables in advanced-stage MF/SS patients, the following independent predictors for worse outcome were identified: stage IV TNMB, age > 60 years, large-cell transformation in skin, elevated white blood cell count, and elevated lactate dehydrogenase (LDH) [14,15].…”

Innovative Behandlungskonzepte mit Modulation der Mikroumgebung beim kutanen T-Zell-Lymphom

“…Da die CTCL eine besonders seltene und heterogene Gruppe von Erkrankungen sind, ist es erst kürzlich gelungen, zuverlässige prognostische Faktoren zu definieren. Eine univariate Analyse von 10 Variablen bei Patienten MF/SS in fortgeschrittenen Stadien ergab die folgenden unabhängigen Prognosefaktoren für einen ungünstigen Verlauf: TNMB-Stadium IV, Alter >60 Jahre, großzellige Transformation in der Haut, erhöhte Leukozytenzahlen und erhöhter LDH-Wert (Laktatdehydrogenase) [14,15]. In Deutschland werden rund 80% der MF-Fälle in frühen Stadien (I-IIA) diagnostiziert [9], was mit einer hervorragenden Prognose und einer 5-Jahres-Überlebensrate von >80% verbunden ist [1,15].…”

“…Während die Prognose des CTCL in frühen Stadien grundsätzlich gut ist, tritt bei einigen Patienten sehr schnell eine Progression ein, und in einer aktuellen Studie sind 4 unabhängige Faktoren für eine schlechtere Überlebensprognose beschrieben worden: Stadium IV, Alter >60 Jahre, großzellige Transformation und erhöhter LDH-Wert [14]. Bei diesen Patienten hat sich die allogene Transplantation hämatopoetischer Stammzellen (alloSCT) als sinnvolle Therapieoption etabliert.…”

Nodal Involvement by CD30+ Cutaneous Lymphoproliferative Disorders and Its Challenging Differentiation From Classical Hodgkin Lymphoma

“…[2][3][4][5] Advanced-stage MF including systemic lymphadenopathy, however, has a poorer prognosis, and large cell transformation is associated with worse outcomes within this group. 6 …”

“…Patients with C-ALCL generally present with solitary or localized ulcerating tumors or nodules. Dissemination to regional lymph nodes can develop in up to 10% to 14% of patients [3][4][5][6] and likely represents the local spread of tumor cells. Patients presenting with involvement of regional lymph nodes have a good prognosis that is similar to that of patients with only skin lesions.…”