A new clinical score for disease activity in Langerhans cell histiocytosis

被引：71

作者：

Donadieu, J

Piguet, C

Bernard, F

Barkaoui, M

Ouache, M

Bertrand, Y

Ibrahim, H

Emile, IF

Hermine, O

Tazi, A

Genereau, T

Thomas, C

机构：

[1] Hop Trousseau, Serv Hemato Oncol Pediat, F-75012 Paris, France

[2] CHU Limoges, Serv Pediat, Limoges, France

[3] CHU Montpellier, Serv Pediat, Montpellier, France

[4] Hop Debrousse, Serv Hematol Pediat, Lyon, France

[5] CHU Amiens, Serv Hemato Pediat, Amiens, France

[6] Hop A Pare Boulogne, Serv Anatomopathol, Boulogne, France

[7] Hop Necker Enfants Malad, Serv Hematol, Toulouse, France

[8] Hop St Louis, Serv Pneumol, Paris, France

[9] Hop St Antoine, Serv Med Interne, F-75571 Paris, France

[10] CHU Nantes, Unite Hemato Oncol Pediat, F-44035 Nantes, France

来源：

PEDIATRIC BLOOD & CANCER | 2004年 / 43卷 / 07期

关键词：

Langerhans cell histiocytosis; prognostic factors; score; therapeutic evaluation;

D O I：

10.1002/pbc.20160

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Objective. To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH). Method. Scoring system was developed and applied to a database containing information on 612 patients. Results. At diagnosis, the score distribution was highly asymmetrical: the score was between 0 and 2 in 74% of cases, 3-6 in 16%, 7-10 in 39%, and more than 10 in 6%. The 5-year mortality rates were 1, 4.4, and 43.4%, respectively, among patients with initial scores of 0-2, 3-6, and >6. Stability or an increase of the score at 6 weeks was highly predictive of death among patients with initial scores above 6, while score stability had no significant impact on vital outcome among patients with low or moderate scores at diagnosis. Conclusions. This LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow-up and treatment. (C) 2004 Wiley-Liss, Inc.

引用

页码：770 / 776

页数：7

共 16 条

[1] Hematopoietic stem cell transplantation in patients with severe Langerhans cell histiocytosis and hematological dysfunction: Experience of the French Langerhans Cell Study Group [J].