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Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood
Author(s) -
Aricò Maurizio,
Astigarraga Itziar,
Braier Jorge,
Donadieu Jean,
Gadner Helmut,
Glogova Evgenia,
Grois Nicole,
Henter JanInge,
Janka Gritta,
McClain Kenneth L.,
Ladisch Stephan,
Pötschger Ulrike,
Rosso Diego,
Thiem Elfriede,
Weitzman Sheila,
Windebank Kevin,
Minkov Milen
Publication year - 2015
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.13271
Subject(s) - langerhans cell histiocytosis , medicine , histiocytosis , bone marrow , gastroenterology , spleen , pathology , white blood cell , disease
Summary Skeletal involvement is generally, but not universally, characteristic of Langerhans cell histiocytosis ( LCH ). We investigated whether the presence of bone lesions at diagnosis is a prognostic factor for survival in LCH . Nine hundred and thirty‐eight children with multisystem ( MS ) LCH , both high (386 RO +) and low ( RO −) risk, were evaluated for bone lesions at diagnosis. Risk organ ( RO +) involvement was defined as: haematopoietic system (haemoglobin <100 g/l, and/or white blood cell count <4·0 × 10 9 /l and/or platelet count <100 × 10 9 /l), spleen (>2 cm below the costal margin), liver (>3 cm and/or hypoproteinaemia, hypoalbuminaemia, hyperbilirubinaemia, and/or increased aspartate transaminase/alanine transaminase). Given the general view that prognosis in LCH worsens with increasing extent of disease, the surprising finding was that in MS + RO + LCH the probability of survival with bone involvement 74 ± 3% ( n = 230, 56 events) was reduced to 62 ± 4% ( n = 156, 55 events) if this was absent ( P = 0·007). An even greater difference was seen in the subgroup of patients with both liver and either haematopoiesis or spleen involvement: 61 ± 5% survival ( n = 105; 52 events) if patients had bony lesions, versus 47 ± 5% ( n = 111; 39 events) if they did not ( P = 0·014). This difference was retained in multivariate analysis ( P = 0·048). Although as yet unexplained, we conclude that bone involvement at diagnosis is a previously unrecognized favourable prognostic factor in MS + RO + LCH .