Mosterd Klara, van Marion Arienne, van Steensel Maurice A M
Department of Dermatology, University Hospital Maastricht, and GROW Research Institute for Oncology and Developmental Biology, University of Maastricht, The Netherlands.
Int J Dermatol. 2008 Nov;47 Suppl 1:10-2. doi: 10.1111/j.1365-4632.2008.03950.x.
A 5-day-old girl presented with thrombocytopenia, leukopenia, anemia and crusted purpura on the skin. The diagnosis Langerhans' cell histiocytosis (LCH) was suspected on clinical grounds and subsequently confirmed by histopathological examination of a skin biopsy. Cytological examination of a bone marrow aspirate revealed numerous histiocytes, which is suspect for bone-marrow infiltration by LCH. LCH is a condition in which a clonal population of Langerhans' cells accumulates in various tissues, causing tissue damage and/or dysfunction. The prognosis of this disease depends on the age of the patient, the extent of the disease and the presence of vital organ failure. In case of organ dysfunction, systemic chemotherapy is indicated. Although very rare, LCH can be a life-threatening disease. Early diagnosis can improve chances of survival. We briefly discuss diagnostic procedures and treatment.
一名5天大的女婴出现血小板减少、白细胞减少、贫血以及皮肤结痂性紫癜。基于临床情况怀疑诊断为朗格汉斯细胞组织细胞增多症（LCH），随后通过皮肤活检的组织病理学检查得以确诊。骨髓穿刺液的细胞学检查发现大量组织细胞，怀疑为LCH骨髓浸润。LCH是一种朗格汉斯细胞的克隆性群体在各种组织中积聚，导致组织损伤和/或功能障碍的疾病。该疾病的预后取决于患者的年龄、疾病范围以及是否存在重要器官功能衰竭。如果出现器官功能障碍，则需进行全身化疗。尽管非常罕见，但LCH可能是一种危及生命的疾病。早期诊断可提高生存几率。我们简要讨论诊断程序和治疗方法。
