朗格罕细胞组织细胞增生症传统分型与Lavin-Osband分级的相关性及其临床意义

目的 朗格罕细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的传统分型无明确标准,各型间临床表现多有交叉,难以定型。传统分型不能判断临床表现的轻重和预后。而Lavin-Osband分级在此方面具有优越性。分析传统分型与Lavin-Osband分级的相关性,并提出后者对临床治疗和预后的指导意义。方法 研究对象为1992年11月～2003年5月间经诊治的25例LCH患儿。对其进行回顾、随访。每一例均采用传统分型和Lavin-Osband分级两种方法,对每一型的不同分级在临床表现、体征、辅助检查、诊断及预后等方面进行相关性分析。结果 LCH临床表现多样;传统分型中各型与发病年龄密切相关,各型的临床表现多有交叉;LCH-Ⅰ型10例,分级均为Ⅲ、Ⅳ级(100％),其中Ⅳ级8例(80％),Ⅰ级、Ⅱ级为O例,属于低型高级;LCH-Ⅱ型6例,Ⅲ级3例(50％),其余分级各1例(17％);LCH-Ⅲ型4例,Ⅰ、Ⅱ级共3例(75％),Ⅲ级1例(25％);Ⅳ级均为0例;LCH-Ⅳ型5例,Ⅰ级和Ⅱ级共4例(80％),Ⅲ级1例(20％),Ⅳ级0例,属于高型低级;Lavin-Osband分级涉及发病年龄、受累器官数和器官功能,综合了传统分型的特点,且对疾病严重程度直观分级,清楚提示预后。结论 Lavin-Osband分级法简单明确,医师在临床中易于掌握,且与患者临床表现的轻重直接相关,依据分级容易对患者的预后做出判断,并及早给予相关治疗和作出治疗计划,提高患者依从性和生存率。

Clinical significance and relationship between traditional classification and Lavin-Osband criteria in Langerhans cell histiocytosis

OBJECTIVE: To analyze the relationship between traditional classification and Lavin-Osband criteria on Langerhans cell histiocytosis (LCH) and to evaluate the importance of the criteria in clinical management and long-term prognosis of Lavin-Osband criteria. METHODS: From 1992 to 2003, 25 cases of LCH were studied. All cases were divided into four types by traditional classification and graded according to Lavin-Osband criteria simultaneously. All patients were analyzed with clinical manifestation, laboratory test, diagnoses, treatment and prognosis. The case numbers distributed in different Lavin-Osband criteria were summed up, and the advantage of the criteria was analyzed. RESULTS: The clinical manifestations according to the traditional classification (four types) overlapped mostly. The age of onset of the disease, the number of involved organs and the function of organs were all considered in the Lavin-Osband criteria, which also contained the characters of the traditional classification and could classify the cases into different severities. So Lavin-Osband criteria could predict the outcome of LCH. According to the traditional classification there were 10 cases with type I LCH, which was all graded to the criteria III and IV (100%), and there were 8 (80%) of criteria IV and none of criteria I and II. The situation was called "low type high criteria". There were 6 cases belong to LCH-II, of them 3 (50%) graded to the criteria III and 1 to the other criteria (17%), respectively. There were 4 cases with LCH-III, of them 3 (75%) graded to the criteria I and II, 1 (25%) to the criteria III and none to the criteria IV. There were 5 cases with LCH-IV, of them 4 (80%) graded to the criteria I and II, 1 (20%) to the criteria III and none to the criteria IV. This situation was called "high type low criteria". CONCLUSION: The Lavin-Osband criteria seemed to be simple, clear and easy to be handled and related to the severity of the disease, which will simplify the diagnoses, help the early-treatment and judge the prognosis.