Lack of bone lesions at diagnosis is associated with inferior outcome in multisystem langerhans cell histiocytosis of childhood |
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| Authors: | Maurizio Aricò Itziar Astigarraga Jorge Braier Jean Donadieu Helmut Gadner Evgenia Glogova Nicole Grois Jan‐Inge Henter Gritta Janka Kenneth L. McClain Stephan Ladisch Ulrike Pötschger Diego Rosso Elfriede Thiem Sheila Weitzman Kevin Windebank Milen Minkov the Histiocyte Society |
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| Affiliation: | 1. Azienda Sanitaria Provinciale 7, Ragusa, Italy;2. Servicio de Pediatria, Bio Cruces Health Research Institute, Hospital Universitario Cruces, Barakaldo, Bizkaia, Spain;3. Departamento de Pediatria, Universidad del Pais Vasco UPV/EHU, Barakaldo, Bizkaia, Spain;4. Hospital Nacional de Pediatría J. Garrahan, Buenos Aires, Argentina;5. Hospital Trousseau, Paris, France;6. Children's Cancer Research Institute and St. Anna Children's Hospital, Vienna, Austria;7. Childhood Cancer Research Unit, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden;8. Department of Haematology and Oncology, University Medical Centre, Hamburg, Germany;9. Texas Children's Cancer and Hematology Centers, Houston, TX, USA;10. Children's Research Institute, Children's National Medical Center, Washington, DC, USA;11. Hospital de Ni?os Elizalde and Hospital de Clinicas UBA, Buenos Aires, Argentina;12. Hospital for Sick Children, Division of Hematology/Oncology, Toronto, ON, Canada;13. Newcastle University, Newcastle, UK |
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| Abstract: | 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 × 109/l and/or platelet count <100 × 109/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. |
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| Keywords: | langerhans cell histiocytosis multisystem disease bone lesion risk organ |
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