Hydroa-like cutaneous T-cell lymphoma:: A clinicopathologic and molecular genetic study of 16 pediatric cases from Peru
Hydroa-like cutaneous T-cell lymphoma:: A clinicopathologic and molecular genetic study of 16 pediatric cases from Peru
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DOI：
10.1097/00022744-200203000-00002
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发表时间：
2002-03-01
影响因子：
1.6
通讯作者：
Piris, MA
中科院分区：
文献类型：
--
作者：
Barrionuevo, C;Anderson, VM;Piris, MA
水螅样皮肤 T 细胞淋巴瘤（水螅样 CTCL）是一种罕见的儿科恶性肿瘤，预后较差。以水肿为特征的令人印象深刻的皮疹。水泡。溃疡、结痂和疤痕，类似于痘痘水痘，主要见于面部，有时见于四肢。病变由皮肤和皮下组织的淋巴瘤 T 细胞浸润组成，具有不同的胞吐作用和血管中心性。它也被称为水肿性、疤痕性血管炎性脂膜炎和水样淋巴瘤。有人认为与 Epstein-Barr 病毒有关。鉴别诊断包括其他皮肤淋巴瘤，特别是皮肤鼻型 T/自然杀伤细胞淋巴瘤、蕈样肉芽肿。前体T细胞淋巴母细胞淋巴瘤。非特异性外周 T 细胞淋巴瘤、皮肤间变性大细胞淋巴瘤和皮下脂膜炎 T 细胞淋巴瘤，其他鉴别诊断包括炎症性皮肤病和脂膜炎。基于肿瘤疾病研究所提交的一系列 16 个此类病例，本报告的目的不仅是提供对该实体更好的临床病理学了解，而且将其重新评估为恶性肿瘤。男女频率比为1：1。中位年龄为10岁。所有病例均以面部受累为主，表现为水肿、水疱、溃疡。结痂和疤痕。化疗和/或放疗几乎没有益处或没有益处。预后通常很差。淋巴瘤从表皮延伸至皮下组织。频繁进行血管中心和附件周围阵列。淋巴瘤细胞大多为中等大小，细胞核致密深染，核仁不明显，有丝分裂少。发现了少量且可变的炎症背景。淋巴瘤细胞取代了 T 细胞的细胞毒性表型。此外。他们的自然杀伤细胞抗原 CD56 和 CD57 呈阴性。检测的 6 例 Epstein-Barr 病毒原位杂交呈阳性。在研究的四个病例中，T 细胞受体伽马 (TCR-伽马) 显示出单克隆型重排。我们的研究结果表明，水螅样 CTCL 是一种独立的临床病理实体，因此会影响儿童。它应被视为 CTCL 的一个独立子集，并因此包含在淋巴组织肿瘤性疾病的分类中。
Hydroa-like cutaneous T-cell lymphoma (hydroa-like CTCL) is an unusual pediatric malignancy with a poor prognosis. An impressive cutaneous rash characterized by edema. blisters. ulcers, crusts, and scars, resembling hidroa vacciniforme, is seen mainly on the face and sometimes on the extremities. The lesion consists of lymphomatous T-cell infiltration of the skin and subcutis with variable exocytosis and angiocentricity. It has been also called edematous, scarring vasculitic panniculitis and hydroa-like lymphoma. An association with Epstein-Barr virus has been suggested. The differential diagnosis includes other cutaneous lymphomas, particularly the cutaneous nasal type T/natural killer-cell lymphoma, mycosis fungoides. precursor T-cell lymphoblastic lymphoma. nonspecific peripheral T-cell lymphoma, cutaneous anaplastic large cell lymphoma, and subcutaneous panniculitic T-cell lymphoma, Other differential diagnoses are inflammatory dermatopathies and panniculitides. Based on a series of 16 such cases referred to the Institute of Neoplastic Diseases, the objective of this report is not only to provide a better clinicopathologic understanding of this entity but also a reappraisal of it as a malignancy. The male/female frequency ratio was 1: 1. The median age was 10 years old. All cases showed predominant facial involvement with edema, blisters, ulcers. crusts, and scars. Chemotherapy and/or radiotherapy had little or no benefit. The prognosis was usually dismal. The lymphoma extended from the epidermis to the subcutis. with frequent angiocentric and periadnexal array. Lymphoma cells were mostly of intermediate size with dense hyperchromatic nuclei, inconspicuous nucleoli, and infrequent mitosis. A scanty and variable inflammatory background was found. The lymphoma cells displaced T-cell cytotoxic phenotype. In addition. they were negative for the natural killer cell antigens CD56 and CD57. Epstein-Barr virus in situ hybridization was positive in the six case,,, in which it was assayed. T-cell receptor gamma (TCR-gamma) displayed monoclonal-type rearrangement in four cases studied. Our findings indicate that hydroa-like CTCL is an independent clinicopathologic entity that affects children Consequently. it should be considered an independent subset of CTCLs and be included as such in the classification of neoplastic diseases of the lymphoid tissues.