Diamantidis Michael D, Myrou Athena D
Department of Haematology, Aristotle University of Thessaloniki, AHEPA Hospital, Thessaloniki, Greece.
ScientificWorldJournal. 2011 May 5;11:1048-55. doi: 10.1100/tsw.2011.80.
Primary cutaneous anaplastic large-cell lymphoma (PC-ALCL), belonging to the CD30+ T-cell lymphoproliferative disorders (PCLPDs), is a rare T-cell lymphoma, presenting on the skin and characterized by very good prognosis and response to treatment in the majority of cases. Nevertheless, PC-ALCL must be distinguished from secondary skin lesions in systemic ALCL, which confer a poor prognosis, and other CD30+ PCLPDs, reactive conditions, or borderline cases. Given their rarity and heterogeneity, these entities represent diagnostic and therapeutic challenges, thus requiring a multidisciplinary approach and expertise to ensure appropriate diagnosis and management. There are several perils and pitfalls that exist regarding the differential diagnosis, the possible progression, and the treatment of PC-ALCL. Careful staging, correlation of clinical findings with histopathology and immunopathology, and thorough follow-up are essential in order to achieve a correct diagnosis and proper treatment of the disease.
原发性皮肤间变性大细胞淋巴瘤（PC-ALCL）属于CD30+ T细胞淋巴增殖性疾病（PCLPDs），是一种罕见的T细胞淋巴瘤，表现于皮肤，多数病例预后良好且对治疗反应良好。然而，PC-ALCL必须与系统性ALCL中的继发性皮肤病变相鉴别，后者预后较差，还需与其他CD30+ PCLPDs、反应性疾病或临界病例相鉴别。鉴于其罕见性和异质性，这些实体构成了诊断和治疗挑战，因此需要多学科方法和专业知识来确保恰当的诊断和管理。在PC-ALCL的鉴别诊断、可能的进展及治疗方面存在若干风险和陷阱。仔细分期、临床发现与组织病理学和免疫病理学的关联以及全面随访对于正确诊断和恰当治疗该疾病至关重要。
