Pagetoid reticulosis (Woringer-Kolopp disease): An immunophenotypic, molecular, and clinicopathologic study
Pagetoid reticulosis (Woringer-Kolopp disease): An immunophenotypic, molecular, and clinicopathologic study
复制标题
DOI：
10.1038/modpathol.3880088
复制
发表时间：
2000-05-01
期刊：
影响因子：
7.5
通讯作者：
Kohler, S
中科院分区：
文献类型：
--
作者：
Haghighi, B;Smoller, BR;Kohler, S
佩吉样网状细胞增生症 (PR)，也称为沃林格-科洛普病，是皮肤 T 细胞淋巴瘤的一种形式，在组织学检查中表现出显着的亲表皮性。我们介绍了 7 名 PR 患者的组织学、免疫学和分子学结果。患者年龄从33岁到67岁不等。除一名患者出现舌头病变外，所有患者均出现涉及远端四肢的一个或多个厚斑块。非典型淋巴细胞的免疫组织化学染色显示所有病例均具有 T 细胞表型。在四个冷冻病例之一中，肿瘤细胞具有 T 辅助细胞表型（CD4 阳性）。七例中有四例表现出 T 细胞毒性/抑制细胞表型（CD8 阳性）。其余两种情况的 T 细胞子集无法确定。通过 Ki-67 染色表明，分别在七例中的三例和四例中的三例中观察到 CD30 阳性和高生长分数。对我们的三个病例进行的基因型分析显示 T 细胞受体（γ 和/或 β）重排，表明克隆增殖。临床随访时间为15个月至13年。经过切除或局部放射治疗后，七名患者中有四人存活并且没有疾病。一名患者在接受放射治疗以及使用 8-甲氧基补骨脂素和 UVA 的光化疗治疗后复发两次，然后失访。另一位患者的病变自然消退，但 5 年后在同一部位和另一个部位复发。一名患者在电子束治疗后复发。通过放射治疗和局部伤口护理，复发性病变有所改善，但从未完全消退，患者死于无关原因。我们的研究结果表明，PR 是一种独特的临床病理学实体，与单病灶蕈样肉芽肿不同，表现出缓慢的病程。该疾病是一种克隆性皮肤 T 细胞淋巴瘤，具有相对一致的临床和组织病理学结果，但具有异质性免疫表型特征。
Pagetoid reticulosis (PR), also known as Woringer-Kolopp disease, is a form of cutaneous T-cell lymphoma that demonstrates striking epidermotropism on histologic examination. We present the histologic, immunologic, and molecular findings for seven patients who had PR. The patients ranged in age from 33 to 67 years. AU patients presented with one or several thick plaques involving the distal extremities except for one patient, who presented with a tongue lesion. Immunohistochemical staining of the atypical lymphoid cells demonstrated a T-cell phenotype in all cases. In one of four frozen cases, the neoplastic cells were of T-helper cell phenotype (CD4 positive). Four of seven cases demonstrated a T-cytotoxic/suppressor cell phenotype (CD8 positive). The T-cell subset for the remaining two cases could not be determined. CD30 positivity and a high growth fraction as indicated by staining with Ki-67 were seen in three of seven and three of four cases, respectively. Genotypic analysis performed on three of our cases revealed T-cell receptor (gamma and/or beta) rearrangement, indicating a clonal proliferation. The clinical follow-up ranged from 15 months to 13 years. Four of seven patients are alive and free of disease after treatment with excision or local irradiation. One patient relapsed twice after treatment with radiation and photochemotherapy with 8-methoxypsoralen and UVA and was then lost to follow-up. The lesions of another patient resolved spontaneously but recurred at the same and in an additional site 5 years later. One patient recurred after electron beam therapy. The recurrent lesion improved with radiation therapy and local wound care but never resolved completely, The patient died of unrelated causes. Our findings suggest that PR is a distinct clinicopathologic entity, separate from unilesional mycosis fungoides, demonstrating a slow disease course. The disease is a clonal cutaneous T-cell lymphoma with relatively consistent clinical and histopathologic findings but a heterogeneous immunophenotypic profile.