Department of Dermatology and Skin Surgery, Boston University School of Medicine and Roger Williams Medical Center, Providence, Rhode Island 02908, USA.
Oncology (Williston Park). 2009 Nov 30;23(13):1158-64.
Primary cutaneous CD30+ T-cell lymphoproliferative disorders (PCLPDs) are the second most common type of cutaneous T-cell lymphoma. These disorders comprise a spectrum of clinically benign lymphomatoidpapulosis (LyP) and primary cutaneous anaplastic large-cell lymphoma (ALCL). The peak incidence of LyP is in the 5th decade of life, and the incidence of primary cutaneous ALCL peaks in the 6th decade, but children are also affected. Both LyP and primary cutaneous ALCL have an excellent prognosis. However, LyP is associated with development of malignant lymphoma (mycosis fungoides, Hodgkin lymphoma, or ALCL) in 20% of cases, and also with an increased risk of non-lymphoid cancers. The diagnosis of LyP is difficult and often delayed. Primary cutaneous ALCL must be distinguished from secondary skin lesions in systemic ALCL, which confer a poor prognosis. Correlation of clinical findings with histopathology and immunopathology (stains for ALK kinase, epithelial membrane antigen, and cutaneous lymphocyte antigen) are important to achieve a correct diagnosis. When a diagnosis of CD30+ PCLPD is established, minimal clinical staging is required. Low-dose methotrexate (10-25 mg weekly) is the most effective therapy for PCLPD but is usually reserved for aggressive cases of LyP and multifocal lesions of cutaneous ALCL Many patients with LyP can be followed expectantly, with special attention to changes in character of the skin lesions or development of lymphadenopathy. Patients with localized cutaneous ALCL can be treated with irradiation. Extracutaneous spread of disease is an indication for multiagent chemotherapy. Other treatment alternatives are discussed.
原发性皮肤 CD30+ T 细胞淋巴增生性疾病（PCLPD）是第二类最常见的皮肤 T 细胞淋巴瘤。这些疾病包括一系列临床良性的蕈样肉芽肿（LyP）和原发性皮肤间变性大细胞淋巴瘤（ALCL）。LyP 的发病高峰在 50 岁左右，而原发性皮肤 ALCL 的发病高峰在 60 岁左右，但儿童也会受到影响。LyP 和原发性皮肤 ALCL 的预后均良好。然而，20%的 LyP 患者会发展为恶性淋巴瘤（蕈样真菌病、霍奇金淋巴瘤或 ALCL），同时也会增加非淋巴样癌症的风险。LyP 的诊断困难且常常延迟。原发性皮肤 ALCL 必须与系统性 ALCL 的继发性皮肤损伤相鉴别，后者预后较差。临床发现与组织病理学和免疫病理学（ALK 激酶、上皮膜抗原和皮肤淋巴细胞抗原染色）的相关性对于正确诊断至关重要。当诊断为 CD30+ PCLPD 时，仅需要进行最低限度的临床分期。低剂量甲氨蝶呤（每周 10-25mg）是 PCLPD 最有效的治疗方法，但通常仅用于 LyP 侵袭性病例和皮肤 ALCL 的多发性病变。许多 LyP 患者可以进行密切观察，特别注意皮肤病变性质的变化或淋巴结病的发展。局限性皮肤 ALCL 患者可以接受放疗。疾病向皮肤外播散是进行多药化疗的指征。其他治疗选择也在讨论中。
