Details

Autor(en) / Beteiligte

• Donadieu, Jean
• Piguet, C.
• Bernard, F.
• Barkaoui, M.
• Ouache, M.
• Bertrand, Y.
• Ibrahim, H.
• Emile, J.F.
• Hermine, O.
• Tazi, A.
• Genereau, T.
• Thomas, C.

Titel

A New clinical score for disease activity in Langerhans cell histiocytosis

Ist Teil von

• Pediatric Blood & Cancer, 2004-12, Vol.43 (7), p.770-776

Ort / Verlag

Hoboken: Wiley Subscription Services, Inc., A Wiley Company

Erscheinungsjahr

2004

Link zum Volltext

Quelle

Wiley-Blackwell Full Collection

Beschreibungen/Notizen

• 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 3%, 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. © 2004 Wiley‐Liss, Inc.