Clinicopathologic, immunophenotypic, and molecular characterization of primary cutaneous follicular B-cell lymphoma.
Clinicopathologic, immunophenotypic, and molecular characterization of primary cutaneous follicular B-cell lymphoma.
复制标题
原发性皮肤滤泡 B 细胞淋巴瘤的临床病理学、免疫表型和分子特征。
作者：
R. Bergman;Paul J. Kurtin;L. Gibson;P. Hull;T. Kimlinger;A. Schroeter
目的
确定原发性滤泡性皮肤B细胞淋巴瘤（CBCL）的临床病理学、免疫表型和分子特征（根据修订的欧美淋巴瘤分类）。
设计
一项病历回顾性调查，一项存档活检标本的免疫组织化学研究。以及对1987年至1997年所有滤泡中心淋巴瘤-滤泡性（FCL-F）原发性CBCL患者的保存DNA进行分子研究。
设置
学术医疗中心的单中心专科门诊。
患者
21例患者（占所有新发原发性CBCL病例的68%），包括14例男性和7例女性（年龄范围：33-88岁;平均年龄：55岁）。
结果
头颈部是最常见的原发部位。治疗后，复发相对频繁，但随访1.0至11.3年（平均6.3年）的总死亡率为4.8%。免疫组化分析B和T细胞谱系有助于增强滤泡样结构。CD 10、bcl-2和CD 43在19例中分别有9例（47%）、4例（21%）和2例（13%）表达。免疫组织化学检测胞浆免疫球蛋白轻链，使用蒸在EDTA作为抗原修复技术，是成功的12（71%）的17例。IG重链基因重排，使用Southern印迹技术，检测克隆性17（94%）的18例。bcl-2基因重排在15例原发性皮肤FCL-F病例中仅2例（13%）检测到，而在12例原发性淋巴结FCL-F病例中有9例（75%）检测到（P = 0.002）。
结论
原发性皮肤FCL-F是CBCL的一种相对常见的亚型，病程相对缓慢。除了bcl-2表达率低和bcl-2基因重排率低外，它与原发性淋巴结FCL-F有许多共同特征。
OBJECTIVE
To determine the clinicopathologic, immunophenotypic, and molecular characteristics of primary follicular cutaneous B-cell lymphoma (CBCL) as defined by the revised European-American lymphoma classification.
DESIGN
A retrospective survey of the medical records, an immunohistochemical study of archival biopsy specimens. and molecular studies of preserved DNA of all patients with follicle center lymphoma-follicular (FCL-F) primary CBCL from 1987 to 1997.
SETTING
A single-center outpatient specialty clinic at an academic medical center.
PATIENTS
Twenty-one patients (68% of all new primary CBCL cases), including 14 men and 7 women (age range, 33-88 years; mean, 55 years).
RESULTS
The head and neck region was the most frequent primary site. Following treatment, recurrences were relatively frequent, but the overall mortality rate during 1.0 to 11.3 years (mean, 6.3 years) of follow-up was 4.8%. Immunohistochemical analysis for B- and T-cell lineages was helpful in enhancing the folliclelike structures. CD10, bcl-2, and CD43 were expressed by the neoplastic cells in 9 (47%) of 19 cases, 4 (21%) of 19 cases, and 2 (13%) of 16 cases, respectively. Immunohistochemical detection of cytoplasmic immunoglobulin light chains, using steaming in EDTA as the antigen-retrieval technique, was successful in 12 (71%) of 17 cases. The Ig heavy-chain gene rearrangements, using the Southern blot technique, detected clonality in 17 (94%) of 18 cases. The bcl-2 gene rearrangements were detected in only 2 (13%) of 15 of the primary cutaneous FCL-F cases, compared with 9 (75%) of 12 of the primary nodal FCL-F cases (P =.002).
CONCLUSIONS
Primary cutaneous FCL-F is a relatively common subtype of CBCL, with a relatively indolent course. It has many features in common with primary nodal FCL-F, except for low rates of bcl-2 expression and bcl-2 gene rearrangements.
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3.5
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YUNIS, JJ;OKEN, MM;THEOLOGIDES, A
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