A CASE OF WORINGER-KOLOPP DISEASE WITH KI-1 (CD30)+ CYTOTOXIC SUPPRESSOR CELLS
A CASE OF WORINGER-KOLOPP DISEASE WITH KI-1 (CD30)+ CYTOTOXIC SUPPRESSOR CELLS
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DOI：
10.1001/archderm.128.4.526
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发表时间：
1992-04-01
影响因子：
--
通讯作者：
WARNKE, R
中科院分区：
文献类型：
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作者：
SMOLLER, BR;STEWART, M;WARNKE, R
背景。- Woringer-Kolopp（W-K）病是一种罕见的，局部的，组织学恶性，但临床惰性淋巴组织增生性疾病。大多数作者认为W-K病是蕈样肉芽肿的一种变异。然而，最近的一个病例表明，W-K病可能代表一系列淋巴组织增生性疾病，可能与蕈样霉菌病无关。 在斯坦福大学（加利福尼亚州）大学医院发现一例40年局限性皮疹病史的患者，其特征为明显的非典型亲表皮淋巴细胞。淋巴细胞主要是CD 30+细胞毒性/抑制性T细胞，这是以前在W-K病中未描述的免疫表型。基因型分析揭示了克隆restriction。结论。 本病例的发现，沿着文献中所有病例的回顾，提示W-K病可能是一个具有统一的临床和组织学表现的实体，但具有明显的免疫表型异质性的恶性外观的非典型细胞。一些病例显示免疫表型相似蕈样肉芽肿。然而，在几乎一半的病例中，包括这里介绍的病例，免疫表型差异超过了相似性。
Background.- Woringer-Kolopp (W-K) disease is a rare, localized, histologically malignant, but clinically indolent lymphoproliferative disorder. Most authors have regarded W-K disease as a variant of mycosis fungoides. However, a recent case suggests that W-K disease may represent a spectrum of lymphoproliferative disorders that may not be related to mycosis fungoides.Observations.- A patient with a 40-year history of a localized cutaneous eruption characterized by markedly atypical epidermotropic lymphocytes was seen at Stanford (Calif) University Hospital. The lymphocytes were predominantly CD30+ cytotoxic/suppressor T cells, an immunophenotype not previously described in W-K disease. Genotype analysis revealed a clonal rearrangement.Conclusions.- The findings in our patient, along with a review of all cases previously reported in the literature, suggest that W-K disease may bean entity with a uniform clinical and histologic presentation, but one with marked immunophenotypic heterogeneity of the malignant-appearing atypical cells. Some cases showed immunophenotypic similarities to mycosis fungoides. However, in almost half of the reviewed cases, including the one presented here, the immunophenotypic differences exceeded the similarities.