Baylor College of Medicine, Houston, TX, USA.
Am J Clin Dermatol. 2011 Dec 1;12(6):389-401. doi: 10.2165/11590390-000000000-00000.
Primary cutaneous small- to medium-sized CD4+ pleomorphic T-cell lymphoma (PCSM-TCL) is a rare and provisional subcategory of cutaneous T-cell lymphoma, associated with a favorable prognosis. We present five cases of PCSM-TCL and review the literature to address questions surrounding its diagnosis and treatment.
A retrospective review was conducted of 353 patients with non-mycosis fungoides cutaneous lymphomas evaluated at the M.D. Anderson Cancer Center Cutaneous Lymphoma Clinic over 10 years. A retrospective search of the English literature using PubMed was conducted to identify additional cases.
Only five patients had a diagnosis of PCSM-TCL. Three patients had unique multifocal presentations that responded well to topical therapy or excision. One patient with localized disease initially treated with radiation therapy had an aggressive relapse requiring systemic therapy. One patient had an isolated nodule that fully resolved with excision. The review of the English literature found 232 additional cases. The presentation of PCSM-TCL was heterogeneous and included solitary/localized papules, nodules, plaques, and tumors. Multiple differential diagnoses were considered and PCSM-TCL diagnosis was based on histopathologic features and clinical correlation. Treatment of solitary/localized lesions included excision or radiation therapy. Multifocal lesions were uncommon and associated with an aggressive course requiring systemic chemotherapy.
The heterogeneous presentation, variable histologic overlap with other lymphoproliferative disorders, and uncertain prognosis of PCSM-TCL creates diagnostic and management dilemmas for clinicians. For treatment, a distinction should be made between indolent and aggressive subtypes. Further investigation is needed to determine if PCSM-TCL is indeed a distinct lymphoma category and, additionally, if aggressive subtypes should remain part of the same diagnostic category.
原发性皮肤中小 CD4+多形性 T 细胞淋巴瘤（PCSM-TCL）是一种罕见的暂定皮肤 T 细胞淋巴瘤亚型，预后良好。我们报告了 5 例 PCSM-TCL，并回顾了文献，以解决其诊断和治疗方面的问题。
对 10 年来在 M.D.安德森癌症中心皮肤科淋巴瘤诊所评估的 353 例非蕈样真菌病皮肤淋巴瘤患者进行了回顾性分析。通过 PubMed 对英文文献进行了回顾性检索，以确定其他病例。
仅有 5 例患者被诊断为 PCSM-TCL。3 例患者有独特的多灶性表现，对局部治疗或切除术反应良好。1 例局限性疾病患者最初接受放射治疗后，病情迅速恶化，需要全身治疗。1 例孤立性结节经切除后完全消退。对英文文献的回顾发现了 232 例其他病例。PCSM-TCL 的表现呈异质性，包括孤立/局限性丘疹、结节、斑块和肿瘤。考虑了多种鉴别诊断，PCSM-TCL 的诊断基于组织病理学特征和临床相关性。孤立/局限性病变的治疗包括切除或放射治疗。多灶性病变不常见，且与侵袭性病程相关，需要全身化疗。
PCSM-TCL 的异质性表现、与其他淋巴增生性疾病的组织学重叠以及不确定的预后给临床医生带来了诊断和管理上的难题。对于治疗，应区分惰性和侵袭性亚型。需要进一步研究以确定 PCSM-TCL 是否确实是一种独特的淋巴瘤类别，以及侵袭性亚型是否应仍然属于同一诊断类别。
