Korean J Hematol.  2011 Sep;46(3):186-191. 10.5045/kjh.2011.46.3.186.

New clinical score for disease activity at diagnosis in Langerhans cell histiocytosis

• Choi WI
• Jeong YC
• Kim SY
• Kim SD
• Pribis JP
• Kim HJ
• Koh KN
• Im HJ
• Lee YH
• Seo JJ

• KMID: 2252002
• DOI: http://doi.org/10.5045/kjh.2011.46.3.186

Abstract

BACKGROUND
The clinical presentation and course of Langerhans cell histiocytosis (LCH) are variable, ranging from an isolated, spontaneously remitting bone lesion to multisystem disease with risk organ involvement. Treatment of LCH ranges from a wait-and-see attitude to intensive multidrug therapy and, in some cases, bone marrow transplantation. It is necessary to develop an objective score for assessing disease activity in patients with LCH. We propose a new clinical scoring system to evaluate disease activity at diagnosis that can predict the clinical outcomes of LCH and correlate it with clinical courses.
METHODS
Clinical data, obtained from children diagnosed with LCH at Asan Medical Center and Hanyang University Hospital between March 1998 and February 2009, were studied retrospectively. The scoring system was developed according to the basic biological data, radiological findings, and physical findings and applied to a database containing information on 133 patients.
RESULTS
The median age of the 133 patients (74 male, 59 female) was 52 months (range, 0.6-178 months), and LCH was diagnosed based on CD1a positivity. At diagnosis, the score distributions were highly asymmetrical: the score was between 1 and 2 in 75.9% of cases, 3-6 in 15.8%, and greater than 6 in 8.3%. Initial scores above 6 were highly predictive of reactivation and late complications.
CONCLUSION
This new LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow-up.

Keyword

Histiocytosis; Langerhans cells; Disease activity; Clinical score

MeSH Terms

Bone Marrow Transplantation
Child
Follow-Up Studies
Histiocytosis
Histiocytosis, Langerhans-Cell
Humans
Langerhans Cells
Male
Retrospective Studies

Figure

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  Fig. 1 The clinical score distribution at initial diagnosis. Scores between 1 and 2 were assigned to 75.9% (N=101) of cases, between 3 and 6 to 15.8% (N=21), and more than 6 to 8.3% (N=11). The number of patients with a score of 1 or 2 was significantly higher than the other groups.

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  Fig. 2 The age distribution of 3 clinical score categories. In the group with scores of 1 or 2, the number of patients who were less than 2 years of age was higher than in the group with scores higher than 3. However, this difference did not reach statistical significance.

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  Fig. 3 The curve of reactivation free survival rate. The reactivation-free survival curves during follow-up in the high score group (>6) was significantly lower than in groups with lower scores.

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