Ju Elizabeth, Adigun Chris, Dunphy Cherie, Gold Stuart, Morrell Dean S
North Carolina University School of Medicine, Chapel Hill, North Carolina 27516, USA.
Pediatr Dermatol. 2012 Jul-Aug;29(4):498-503. doi: 10.1111/j.1525-1470.2011.01465.x. Epub 2011 Oct 4.
Anaplastic large cell lymphoma (ALCL) accounts for 10% to 30% of all childhood lymphomas and approximately 5% of all non-Hodgkin's lymphoma. ALCL is considered to be a T-cell non-Hodgkin's lymphoma that can be divided into two major groups with distinct genetic, immunophenotypic, and clinical behaviors. The first group consists of a spectrum of CD30+ T-cell lymphoproliferative disorders that include primary cutaneous ALCL (C-ALCL) and lymphomatoid papulosis. The second group is systemic ALCL (S-ALCL), which is further divided into two subgroups: anaplastic lymphoma kinase positive (ALK+) and ALK-negative. Between 30% and 60% of S-ALCL express ALK, which is usually the result of a t(2;5) translocation that correlates with onset in the first three decades of life, male predominance, and good prognosis. Although morphologically similar, ALK- ALCL shows varied clinical behaviors and immunophenotypes; is commonly seen in older age groups, with a peak incidence in the sixth decade of life with no preference as to sex; and has an overall poorer prognosis. We present a case of CD30+, ALK- S-ALCL in a 7-year-old girl.
间变性大细胞淋巴瘤（ALCL）占所有儿童淋巴瘤的10%至30%，约占所有非霍奇金淋巴瘤的5%。ALCL被认为是一种T细胞非霍奇金淋巴瘤，可分为两个主要组，具有不同的遗传、免疫表型和临床行为。第一组包括一系列CD30+T细胞淋巴增殖性疾病，包括原发性皮肤ALCL（C-ALCL）和淋巴瘤样丘疹病。第二组是系统性ALCL（S-ALCL），进一步分为两个亚组：间变性淋巴瘤激酶阳性（ALK+）和ALK阴性。30%至60%的S-ALCL表达ALK，这通常是t（2；5）易位的结果，与生命的前三十年发病、男性 predominance和良好预后相关。尽管形态学上相似，但ALK-ALCL表现出不同的临床行为和免疫表型；常见于老年人群，在第六个十年发病率最高，无性别偏好；总体预后较差。我们报告一例7岁女孩的CD30+、ALK-S-ALCL病例。 （注：原文中“male predominance”这里的“predominance”可能有误，推测可能是“predominance”，意为“男性占优势” ，翻译时按此推测翻译，供你参考。）
