University of Minnesota Medical School, Minneapolis, MN, USA.
Pediatr Blood Cancer. 2012 Jun;58(6):998-9. doi: 10.1002/pbc.23313. Epub 2011 Sep 15.
Langerhans cell histiocytosis (LCH) is a proliferative disorder of dendritic cells which has evaded efforts to clearly define pathogenesis, diagnostic criteria, and therapeutic response markers. Strides have been made at classification with the recent development of a quantified score of disease severity. Splenic involvement is an indicator of poor prognosis, with spleen size its surrogate marker in evaluation and scoring. We describe a case of pediatric LCH with progressive splenomegaly despite treatment, which when examined at splenectomy revealed no LCH involvement but extramedullary hematopoiesis. These findings thus challenge our understanding of splenomegaly as a marker of disease.
朗格汉斯细胞组织细胞增生症（LCH）是树突状细胞的增生性疾病，其发病机制、诊断标准和治疗反应标志物的定义仍不明确。随着疾病严重程度量化评分的发展，在分类方面取得了进展。脾受累是预后不良的指标，脾肿大是评估和评分的替代标志物。我们描述了一例儿童 LCH 患者，尽管接受了治疗，但脾肿大仍进行性加重，脾切除术检查显示无 LCH 受累，但有髓外造血。这些发现因此挑战了我们对脾肿大作为疾病标志物的理解。
