Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type

Abstract

To describe clinicopathologic features and to identify prognostic factors in a large series of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT), as defined in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas.Design: Retrospective multicenter study from the French Study Group on Cutaneous Lymphomas.Setting: Nineteen departments of dermatology in 10 regions of France.Patients: Sixty patients with a PCLBCL LT included in the registry of the French Study Group on Cutaneous Lymphomas.Main Outcome Measures: Age, sex, outcome, therapy, B symptoms, cutaneous extent, number of lesions, location (leg vs nonleg), serum lactate dehydrogenase level, and MUM-1 and Bcl-2 expression were recorded. Diseasespecific survival was used as the main end point. Prognostic factors were identified using a Cox proportional hazards model.Results: Primary cutaneous diffuse large B-cell lymphoma, leg type is characterized by a predilection for the leg (72%), a high proportion of Bcl-2 expression (85%), an advanced age at onset (mean age, 76 years), and frequent relapses and extracutaneous dissemination. The overall 5-year disease-specific survival rate was 41%. Location on the leg and multiple skin lesions were predictive of death in multivariate analysis. Although no variable related to therapy was significantly associated with survival, patients recently treated with combinations of anthracycline-containing chemotherapies and rituximab had a more favorable shortterm outcome.Conclusions: Primary cutaneous diffuse large B-cell lymphoma, leg type is a distinct entity with a poor prognosis, particularly in patients with multiple tumors on the legs. Despite the advanced age of many patients, the prognosis could be improved with combinations of anthracycline-containing chemotherapies and rituximab.

Isolated Limb Perfusion with Melphalan for the Treatment of Intractable Primary Cutaneous Diffuse Large B-Cell Lymphoma Leg Type

“…Different treatment options have been proposed such as polychemotherapy [9], surgery [10], radiation [2] and immunotherapy [11]. There are currently no uniform international guidelines [2], as each treatment modality gives controversial results [12]. However, a recent European consensus has designed the R-CHOP regimen as standard of care [13].…”

“…Accordingly, isolated limb perfusion seems to be as effective as systemic therapy after recurrence. Recurrence is in 2/3 of all cases restricted to the skin and most frequently to the leg in the case of DLBCL leg type [12]. Due to this limited recurrence pattern, local treatment might prove effective, as was seen in other cutaneous NHL [18].…”

Primary Cutaneous Diffuse Large B-Cell Lymphoma (Leg Type) Presenting as Necrotising Fasciitis

“…An elevated serum lactate deydrogenase has been identified in 11.7% of cases [14]. Approximately 25% of patients will develop extracutaneous disease at a mean time of 22 months while rarely lesions may present in a primary location other than the leg and carry a better prognosis [13,15].…”

Rezidiv eines systemischen, diffus-großzelligen B-Zell-Lymphoms unter dem Bild eines primär kutanen, diffus-großzelligen B-Zell-Lymphoms, Leg Type

“…Aufgrund des häufig vorkommenden extrakutanen Befalls ist die Prognose mit einer 5-Jahres-Überlebensrate von 55 % erheblich schlechter als bei den indolenten kutanen B-Zell-Lymphomen [5]. In der aktuellen WHO-EORTC-Lymphom-Klassifikation der kutanen Lymphome werden neben dem PCDLBL, LT, auch noch andere Formen des primär kutanen, diffus-großzelligen B-Zell-Lymphoms aufgeführt (PCDLBL, other) [1,2,6]. Dabei handelt es sich um diffuse B-Zell-Lymphome, welche sich klinisch, histologisch und phänotypisch nicht eindeutig dem PCDLBL, LT, bzw.…”

“…Dabei handelt es sich um diffuse B-Zell-Lymphome, welche sich klinisch, histologisch und phänotypisch nicht eindeutig dem PCDLBL, LT, bzw. dem primär kutanen Keimzentrumslymphom zuordnen lassen [1,2,6]. …”