Rashidghamat E, Robson A
St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, UK.
Department of Dermatopathology, St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, UK.
Clin Exp Dermatol. 2015 Dec;40(8):839-43. doi: 10.1111/ced.12658. Epub 2015 May 9.
Precursor B-cell lymphoblastic lymphoma (PBLL) is a rare subtype of childhood non-Hodgkin lymphoma (NHL). Most lymphoblastic lymphomas have a T-cell immunophenotype, but a small distinct proportion is of precursor B-cell origin. Skin and bone involvement is seen more commonly in this clinical variant. Primary cutaneous PBLL is rare. We describe an 8-year-old girl who presented with an asymptomatic nodule on the left upper arm. Histopathological features were consistent with pre-B-cell lymphoblastic lymphoma, and staging investigations excluded extracutaneous disease, resulting in a diagnosis of primary cutaneous PBLL. The child was started on induction chemotherapy, UKALL 2003 regimen B. She developed disseminated varicella zoster virus and died despite treatment. We discuss previously reported cases of primary cutaneous PBLL and their outcomes.
前体B细胞淋巴母细胞淋巴瘤(PBLL)是儿童非霍奇金淋巴瘤(NHL)的一种罕见亚型。大多数淋巴母细胞淋巴瘤具有T细胞免疫表型,但一小部分具有明显特征的病例起源于前体B细胞。在这种临床变体中,皮肤和骨骼受累更为常见。原发性皮肤PBLL很少见。我们描述了一名8岁女孩,她左上臂出现一个无症状结节。组织病理学特征与前B细胞淋巴母细胞淋巴瘤一致,分期检查排除了皮肤外疾病,从而诊断为原发性皮肤PBLL。该患儿开始接受诱导化疗,采用UKALL 2003方案B。她发生了播散性水痘带状疱疹病毒感染,尽管接受了治疗仍死亡。我们讨论了先前报道的原发性皮肤PBLL病例及其结局。