Geissmann F, Thomas C, Emile J F, Micheau M, Canioni D, Cerf-Bensussan N, Lazarovits A I, Brousse N
Unité d'Immunologie et d'Hématologie, Hôpital Necker-Enfants Malades, Université René Descartes, Paris, France.
J Pediatr. 1996 Dec;129(6):836-45. doi: 10.1016/s0022-3476(96)70027-6.
Langerhans cell histiocytosis (LCH) is a rare disease with a wide clinical spectrum. Although little is known of gastrointestinal involvement in LCH, it may be a major clinical problem. We investigated clinical, pathologic, and immunohistochemical features of digestive tract LCH involvement in children.
Selection criteria consisted of the presence of LCH with digestive symptoms, and histologic confirmation of gastrointestinal involvement. Seven children (2%) met the criteria among 348 cases of LCH in a French national retrospective survey from 1983 to 1993. Two children whose LCH was diagnosed in 1994 were also selected.
Nine children with LCH and digestive tract involvement were studied. Clinical features at presentation included skin (9/9) and mucosal (4/9) involvement, failure to thrive (5/9), diarrhea (7/9), bloody stools (4/7), vomiting (4/9), and hypoalbuminemia (8/9). Five of the nine children died; factors associated with a poor prognosis included young age, organ dysfunction (stage 4), and need for parenteral nutrition. Unlike control biopsy specimens, LCH cells of children with digestive tract involvement disclosed expression of the mucosal homing receptor integrin alpha 4 beta 7 on frozen skin and digestive tract biopsy specimens.
Cutaneous, mucosal, and digestive tract involvement in LCH is a clinicopathologic entity. The prognosis and treatment of LCH depend on the extent of the disease; therefore the treatment of these disseminated forms should not be delayed. Thus children with cutaneous LCH and digestive symptoms should undergo digestive tract biopsies. Studies of homing receptors may contribute to our understanding of the mechanisms of dissemination in LCH.
朗格汉斯细胞组织细胞增多症（LCH）是一种临床谱广泛的罕见疾病。尽管对LCH累及胃肠道的情况了解甚少，但这可能是一个主要的临床问题。我们研究了儿童消化道LCH累及的临床、病理和免疫组化特征。
选择标准包括存在LCH且有消化系统症状，以及胃肠道累及的组织学证实。在1983年至1993年法国一项全国性回顾性调查的348例LCH病例中，有7名儿童（2%）符合标准。还选择了1994年诊断为LCH的2名儿童。
对9例LCH累及消化道的儿童进行了研究。就诊时的临床特征包括皮肤受累（9/9）、黏膜受累（4/9）、发育不良（5/9）、腹泻（7/9）、血便（4/7）、呕吐（4/9）和低白蛋白血症（8/9）。9名儿童中有5名死亡；与预后不良相关的因素包括年龄小、器官功能障碍（4期）和需要肠外营养。与对照活检标本不同，消化道受累儿童的LCH细胞在冰冻皮肤和消化道活检标本上显示黏膜归巢受体整合素α4β7的表达。
LCH累及皮肤、黏膜和消化道是一种临床病理实体。LCH的预后和治疗取决于疾病的程度；因此，对于这些播散性形式的治疗不应延迟。因此，有皮肤LCH和消化道症状的儿童应接受消化道活检。对归巢受体的研究可能有助于我们理解LCH的播散机制。
