McCarty M J, Vukelja S J, Sausville E A, Perry J J, James W D, Jaffe E S, Weiss R B
Department of Medicine, Brooke Army Medical Center, Fort Sam Houston, Texas.
Cancer. 1994 Dec 1;74(11):3051-8. doi: 10.1002/1097-0142(19941201)74:11<3051::aid-cncr2820741124>3.0.co;2-p.
Lymphomatoid papulosis (LyP) is an uncommon disorder characterized by recurrent papulonodular cutaneous lesions that last from 4 to 5 weeks and often heal with hypopigmented or hyperpigmented scarring. Prognosis is varied, 10%-20% of patients have associated lymphomas: mycosis fungoides, T-cell immunoblastic lymphoma, or Hodgkin's disease, which can precede, occur simultaneously with, or follow the diagnosis of LyP. Anaplastic large cell lymphoma (ALCL) is histologically and phenotypically similar to LyP and also appears as part of this disease spectrum. Recent reports analyzing immunophenotype and T-cell receptor gene rearrangements in patients with both LyP and lymphoma suggest that they are derived from an identical T-cell clone, in the rare cases studied.
The case histories of two patients with LyP in whom ALCL involving the skin and lymph nodes subsequently developed are presented.
Intensive treatment with combination chemotherapy resulted in complete remission of ALCL in both patients, followed by the recurrence of LyP. A spontaneous remission of LyP occurred in the initial patient described, whereas the second patient suffered recurrences of both LyP and ALCL despite therapy.
The case histories presented illustrate the immunophenotypic and morphologic similarities of ALCL and LyP, and the difficulties in distinguishing between them. Both entities can occur in a single patient, as shown by this report, supporting a close relationship between these processes. However, different clinical behavior and response to therapy are apparent, which connote a fundamental difference in the biologies of these neoplastic disorders. A review of the literature concerning the association between these entities is provided.
淋巴瘤样丘疹病（LyP）是一种罕见的疾病，其特征为反复出现的丘疹结节性皮肤损害，持续4至5周，愈合后常留有色素减退或色素沉着性瘢痕。预后各异，10% - 20%的患者伴有淋巴瘤：蕈样肉芽肿、T细胞免疫母细胞淋巴瘤或霍奇金病，这些淋巴瘤可在LyP诊断之前、同时或之后出现。间变性大细胞淋巴瘤（ALCL）在组织学和表型上与LyP相似，也出现在这一疾病谱中。最近对同时患有LyP和淋巴瘤患者的免疫表型及T细胞受体基因重排进行分析的报告表明，在少数研究病例中，它们源自同一个T细胞克隆。
报告了2例患有LyP的患者，随后均发生了累及皮肤和淋巴结的ALCL。
联合化疗的强化治疗使2例患者的ALCL完全缓解，随后LyP复发。首例报告的患者LyP自发缓解，而第二例患者尽管接受了治疗，LyP和ALCL仍复发。
所报告的病例说明了ALCL和LyP在免疫表型和形态学上的相似性，以及区分它们的困难。如本报告所示，这两种疾病可发生在同一患者身上，支持了这些病变之间的密切关系。然而，不同的临床行为和对治疗的反应是明显的，这意味着这些肿瘤性疾病在生物学特性上存在根本差异。本文提供了有关这些疾病之间关联的文献综述。
