CD30+ Lymphoproliferative Disorders of the Skin.
CD30+ Lymphoproliferative Disorders of the Skin.
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CD30 皮肤淋巴增殖性疾病。
DOI：
10.1016/j.hoc.2016.11.006
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发表时间：
2017
期刊：
影响因子：
--
通讯作者：
LeBoeuf,NicoleR
中科院分区：
文献类型：
--
作者：
Sauder,MaxwellB;O'Malley,JohnT;LeBoeuf,NicoleR
原发性皮肤CD 30+淋巴组织增生性疾病包括淋巴瘤样丘疹病（LyP）、原发性皮肤间变性大细胞淋巴瘤（pcALCL）和不确定病例。LyP是一种良性疾病，其特征是反复出现数至数百个丘疹结节，红色或紫色，通常在躯干和四肢上长达20 mm。LyP患者发生继发性恶性肿瘤的风险增加，这些恶性肿瘤可能在LyP诊断之前、期间或之后被诊断出来，因此应接受持续监测。pcALCL的特征是孤立的红色至紫色结节或肿瘤大于20 mm，可发生在身体的任何部位。对于最初表现为ALCL皮肤病变的任何患者，必须排除继发性皮肤ALCL。LyP是良性的，局限于皮肤，可自行消退，5年生存率为100%; pcALCL通常局限于皮肤，对定向治疗有反应，5年生存率超过95%。应避免使用侵入性全身或多药化疗方案。
Primary cutaneous CD30+ lymphoproliferative disorders encompass lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (pcALCL) and indeterminate cases. LyP is a benign disorder characterized by recurrent crops of several to hundreds of papulonodules, red or violaceous in color and measuring up to 20mm, usually on the trunk and extremities. Patients with LyP are at increased risk of a secondary malignancy that may be diagnosed before, during or after the diagnosis of LyP and thus should receive ongoing surveillance. pcALCL is characterized by a solitary red to violaceous nodule or tumor greater than 20mm and may occur anywhere on the body. Secondary cutaneous ALCL must be excluded for any patient initially presenting with a cutaneous lesion of ALCL. LyP is benign, limited to the skin and self-resolves with a 5-year survival rate of 100%; pcALCL is usually limited to the skin and responsive to directed therapies, with a 5-year survival of over 95%. Aggressive systemic or multi-agent chemotherapeutic regimens should be avoided.