CD20+ T-cell Lymphoma Clinicopathologic Analysis of 9 Cases and a Review of the Literature
CD20+ T-cell Lymphoma Clinicopathologic Analysis of 9 Cases and a Review of the Literature
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DOI：
10.1097/pas.0b013e31817d7452
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发表时间：
2008-11-01
影响因子：
5.6
通讯作者：
Ferry, Judith A.
中科院分区：
文献类型：
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作者：
Rahemtullah, Aliyah;Longtine, Janina A.;Ferry, Judith A.
CD20(+) t细胞淋巴瘤（TCL）的罕见病例已被报道，但这种疾病的临床病理谱尚不清楚。通过查阅英语文献，我们确定了9例在我院诊断的CD20(+) TCL和26例其他病例。在目前的病例中，有7名男性（71岁至81岁，中位年龄75岁）和2名女性（36岁和37岁）。5例患者主要表现为淋巴结疾病（3例局限，2例广泛），4例患者表现为纯粹淋巴结外疾病，累及腮腺、皮肤或小肠。CD20在5例中均匀且强烈表达，在4例肿瘤细胞亚群中微弱表达或存在。3例患者CD20(+) T细胞比例随时间变化。3例符合临床病理定义的TCL亚型诊断标准（2例蕈样真菌病；1例肠病型TCL）， 6例为外周TCL，未明确细胞形态、t细胞免疫表型和受累部位。9例中有8例通过分子遗传分析鉴定出克隆t细胞群。在8例临床随访中，5例表现积极，4例在诊断3年内死于疾病（中位生存期：11个月，范围：1至35个月），1例在10个月时复发，1例在最初诊断66个月后死于EBV+ b细胞淋巴瘤（BCL）；其余2例患者存活并接受治疗（随访时间分别为4和18个月）。历史病例表现出类似的临床病理变异性。CD20(+) TCL是罕见的，临床和病理异质性。当CD20在TCL中表达时，其表达可能比正常B细胞暗，提示正常CD20dim(+) t细胞亚群发生了肿瘤转化。CD20(+) TCL的病例中，CD20（+）细胞的比例随着时间的推移而变化，可能反映了肿瘤T细胞对CD20的异常表达，可能作为激活标记物。CD20(+) TCL可能导致诊断困难，特别是在临床和病理上类似BCL的病例中。了解CD20在TCL中表达的不寻常现象，结合仔细的形态学分析、抗体小组的使用和分子遗传学研究，对于避免BCL的误诊是重要的。
Rare cases of CD20(+) T-cell lymphoma (TCL) have been reported, but the clinicopathologic spectrum of this disorder is not known. We identified 9 cases of CD20(+) TCL diagnosed at Our institution and 26 additional cases through a search of the English language literature. Among current cases, there were 7 men (ages 71 to 8 1, median 75 y) and 2 women (ages 36 and 37 y). Five patients presented with predominantly nodal disease (localized in 3 and widespread in 2 cases) and 4 patients presented with purely extranodal disease involving the parotid glands, skin, or small intestine. CD20 was uniformly and strongly expressed in 5 cases and dimly expressed or present on a Subset of neoplastic cells in 4 cases. The proportion of CD20(+) T cells changed over time in 3 cases. Three cases fulfilled diagnostic criteria for clinicopathologically defined Subtypes of TCL (2 mycosis fungoides; 1 enteropathy-type TCL), whereas 6 were peripheral TCL unspecified with variable cytomorphology, T-cell immunophenotype, and sites of involvement. In 8 of 9 cases, a clonal T-cell Population was identified by molecular genetic analysis. Among 8 cases with clinical follow-up, 5 behaved aggressively with death from disease within 3 years of diagnosis in 4 cases (median Survival: 11 mo, range: 1 to 35 mo), and recurrent disease at 10 months in 1 cased 1 patient died of an EBV+ B-cell lymphoma (BCL) 66 months after the original diagnosis; in the remaining 2 cases, patients were alive and undergoing treatment (Follow-up: 4 and 18 mo). Historical cases showed similar clinicopathologic variability. CD20(+) TCL is rare, and clinically and pathologically heterogeneous. When CD20 expression is present in TCL, it may be dimmer than that of normal B cells, suggesting neoplastic transformation of a normal CD20dim(+) T-cell Subset. Cases of CD20(+) TCL in which the proportion of CD20(+) cells changes over time may reflect aberrant expression of CD20, possibly as in activation marker, by neoplastic T cells. CD20(+) TCL may cause diagnostic difficulty, particularly in cases that clinically and pathologically mimic BCL. Knowledge of the unusual phenomenon of CD20 expression in TCL, in conjunction with careful morphologic analysis, the use of a panel of antibodies, and molecular genetic Studies, is important in avoiding a misdiagnosis of BCL.