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Diagnostic and Prognostic Value of BCL2 Rearrangement in 53 Patients With Follicular Lymphoma Presenting as Primary Skin Lesions.

BCL-2基因重排在53例皮肤原发滤泡性淋巴瘤中的诊断和预后价值

Pham-Ledard A,Cowppli-Bony A,Doussau A,Prochazkova-Carlotti M,Laharanne E,Jouary T,Belaud-Rotureau MA,Vergier B,Merlio JP,Beylot-Barry M

Abstract

To study the diagnostic value of BCL2 rearrangement in follicle center lymphoma (FCL) presenting as primary skin lesions, evaluate its prevalence and the prognostic value in primary cutaneous FCL (PCFCL), and assess prognostic factors in PCFCL.
Fifty-three patients with a cutaneous presentation of FCL without a history of nodal lymphoma were selected retrospectively. Clinical and histologic data were collected together with staging and follow-up data. A fluorescence in situ hybridization (FISH) test for BCL2 split probes was performed on skin biopsy specimens.
Initial staging procedures identified 47 PCFCLs and six cases of secondary skin involvement of FCL (SSIFCL). FISH detected seven cases carrying a BCL2 rearrangement: four (8.5%) of 47 PCFCLs and three (50%) of six SSIFCLs. These seven cases coexpressed BCL2 and CD10. In PCFCL, cutaneous relapse rate was 42.6%. A small/medium centrocytic cell population was associated with a higher probability of skin relapse in univariate (P = .008) and multivariate (P = .028) analysis, and BCL2 rearrangement detection was associated with secondary extracutaneous spreading (P = .05).
We observed that BCL2 rearrangement in PCFCL is rare, associated with initial positivity of staging (diagnostic value) or with secondary extracutaneous spreading (prognostic value). In selected cases with BCL2-CD10 coexpression, FISH testing could detect patients with poor outcome and require closer monitoring.

摘要

目的:研究BCL-2基因重排在皮肤原发滤泡性淋巴瘤中的诊断价值,评价它在皮肤原发滤泡性淋巴瘤中的比例和预后价值,评估皮肤原发滤泡性淋巴瘤的预后因素。

收集53例原发皮肤滤泡性淋巴瘤患者,病史回顾均无淋巴结的淋巴瘤病史。收集临床和组织学资料以及随访资料。在这些皮肤活检标本中用BCL-2分离探针做荧光原位杂交检测。

经初步鉴定,有47例原发PCFCLs患者、6例滤泡性淋巴瘤累及皮肤(SSIFCL)的患者。FISH检测有7例存在BCL-2基因重排,其中47例PCFCLs中有4例(8.5%),6例SSIFCL中有3例(50%);这7例均表达BCL2和CD10。在PCFCL中,皮肤病变的复发率是42.6%。单变量方差分析(P=0.008)和多变量方差分析(P=0.028)表明中/小中心细胞聚集和较高的皮肤复发率存在相关性;BCL-2基因重排与再次真皮外扩散有相关性(P=0.05)。

我们注意到PCFCL中BCL-2基因重排的发生是罕见的,与初始分期(诊断价值)和再次真皮外扩散(预后价值)有相关性。在挑选出的BCL2-CD10共表达病例中,FISH检测能发现那些预后不良和需要密切监测的患者。

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