Clinical and pathological spectrum of CD8‐positive cutaneous T‐cell lymphomas

Clinical and pathological spectrum of CD8‐positive cutaneous T‐cell lymphomas
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CD8阳性皮肤T细胞淋巴瘤的临床和病理谱

DOI:
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发表时间:
2002
影响因子:
1.7
通讯作者:
Dan M. Jones
Dan M. Jones
中科院分区:
医学4区
文献类型:
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作者:
D. Lu;Kaushali A. Patel;M. Duvic;Dan M. Jones

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CD8+ T细胞淋巴瘤很少出现在皮肤上。我们描述了18例CD8+皮肤T细胞肿瘤患者的临床和组织学特征,这些患者被分为四组。7例患者既往有长期皮疹病史,其逐渐扩散的表现与CD4+真菌样真菌病(MF)相似。3例患者有长期存在的局部斑块,符合页面样网状病(PR)模式。2例患者表现为红皮病,外周血受累与Sezary综合征(SS)模式一致,临床病程进展迅速。6例患者出现不同大小的皮肤结节,结果不同,其中2例疾病迅速进展,2例惰性复发,另外2例对治疗完全有效。组织学上,18例中有12例表现为表皮性肿瘤浸润,其中3例PR最为明显。突出的附件周浸润11例。与CD4+ MF相似,皮肤归巢抗原(皮肤淋巴细胞抗原:CLA)在16例测试病例中有13例强烈表达。在16例测试病例中,只有3例在大多数肿瘤细胞中发现细胞毒性颗粒蛋白颗粒酶B的表达。我们得出结论,大约一半的CD8+皮肤T细胞淋巴瘤在临床和组织学上类似于CD4+ MF/SS,而与表达CD4的肿瘤相比,CD8+肿瘤表现为离散结节性病变更常见。
CD8+ T‐cell lymphomas presenting in the skin are rare. We describe the clinical and histological features of 18 patients with CD8+ cutaneous T‐cell tumors, which have been divided into four groups. Seven patients had precedent long histories of rashes, which progressively spread in a presentation similar to that of CD4+ mycosis fungoides (MF). Three patients had long‐standing localized plaques consistent with a pagetoid reticulosis (PR) pattern. Two patients presented with erythroderma and had peripheral blood involvement consistent with a Sezary syndrome (SS) pattern and had rapidly progressive clinical courses. Six patients presented with cutaneous nodules of varying sizes and had variable outcomes, with two having rapidly progressive disease, two with indolent recurrences and a further two with complete responses to treatment. Histologically, 12 of the 18 cases showed an epidermotropic tumor infiltrate that was most marked in the three PR cases. Prominent periadnexal infiltration was seen in 11 cases. Similar to CD4+ MF, the skin‐homing antigen, (cutaneous lymphocyte antigen: CLA), was strongly expressed in 13 of 16 tested cases. Expression of the cytotoxic granule protein granzyme B was noted in a majority of tumor cells in only three of 16 tested cases. We conclude that approximately half of CD8+ cutaneous T‐cell lymphomas clinically and histologically resemble CD4+ MF/SS, whereas presentation as discrete nodular lesions are more common in CD8+ tumors as compared to those that express CD4.