朗格汉斯细胞组织细胞增多症中传统分类与拉文-奥斯本标准的临床意义及关系

Ma Li-juan, Shi Xiao-dong, Wang Tian-you, Chen Jing, Liu Rong

Department of Internal Medicine, The Capital Insititute of Pediatrics, Beijing 100020, China.

Zhonghua Er Ke Za Zhi. 2004 Jan;42(1):58-61.

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.

目的

分析朗格汉斯细胞组织细胞增多症（LCH）的传统分类与拉文 - 奥斯本标准之间的关系，并评估拉文 - 奥斯本标准在临床管理及长期预后中的重要性。

方法

研究1992年至2003年期间的25例LCH病例。所有病例按传统分类分为四型，同时依据拉文 - 奥斯本标准进行分级。对所有患者的临床表现、实验室检查、诊断、治疗及预后进行分析。汇总不同拉文 - 奥斯本标准下的病例数，分析该标准的优势。

结果

传统分类（四型）的临床表现大多重叠。拉文 - 奥斯本标准综合考虑了发病年龄、受累器官数量及器官功能，既包含了传统分类的特征，又能将病例分为不同严重程度，从而可预测LCH的预后。按照传统分类，I型LCH有10例，均被分级为标准III级和IV级（100%），其中标准IV级有8例（80%），I级和II级无。此情况称为“低型高标准”。属于LCH-II型的有6例，其中3例（50%）分级为标准III级，1例（17%）分级为其他标准。LCH-III型有4例，其中3例（75%）分级为标准I级和II级，1例（25%）分级为标准III级，无IV级。LCH-IV型有5例，其中4例（80%）分级为标准I级和II级，1例（20%）分级为标准III级，无IV级。此情况称为“高型低标准”。