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Long-term clinical outcome of spinal Langerhans cell histiocytosis in children

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Abstract

Spinal involvement of Langerhans cell histiocytosis (LCH) affects morbidity, but outcomes are not well understood. We analyzed long-term outcomes following uniform treatment at a single institution. Clinical characteristics and outcomes of spinal LCH patients were retrospectively analyzed. Height ratios were calculated using the anterior height of the involved vertebral body on magnetic resonance imaging (MRI) and the expected normal vertebral height. Twenty-two (22.4%) of 98 patients diagnosed with LCH had spinal involvement. The median age at diagnosis was 4.1 (range 0.6–12.3) years. Thirty-one spinal lesions were identified in 22 patients; the thoracic spine (n = 17) was most commonly affected. Eight lesions with minimal collapse, which appeared normal on plain radiography, were detected with MRI. All patients received vinblastine-based chemotherapy. Fourteen (70%) of 20 evaluable vertebral body collapses, including eight severe lesions, showed improvement in vertebral body height at a median follow-up of 6.0 (range 2.8–12.0) years. All traceable patients were alive without disease. Long-term follow-up of vertebral body collapse revealed vertebral height improvement in approximately 70% of spinal LCH patients, even in severe cases. MRI at diagnosis detected spinal lesions earlier with higher sensitivity than plain radiography.

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Abbreviations

LCH:

Langerhans cell histiocytosis

MRI:

Magnetic resonance imaging

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Author information

Author notes

  1. Seong Wook Lee and Hyery Kim contributed equally to this work.

Authors and Affiliations

  1. Department of Pediatrics, Korea University Ansan Hospital, Korea University College of Medicine, Seoul, Korea

    Seong Wook Lee

  2. Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea

    Hyery Kim, Kyung-Nam Koh, Ho Joon Im & Jong Jin Seo

  3. Department of Pediatrics, Kyungpook National University Hospital, Daegu, Korea

    Jin Kyung Suh

  4. Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

    Hee Mang Yoon

Authors
  1. Seong Wook Lee
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  2. Hyery Kim
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  3. Jin Kyung Suh
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Correspondence to Jong Jin Seo.

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Funding

This work was supported by the Research Program funded by the Korea Centers for Disease Control and Prevention (2016E6300200).

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The authors declare that they have no competing interest.

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Lee, S.W., Kim, H., Suh, J.K. et al. Long-term clinical outcome of spinal Langerhans cell histiocytosis in children. Int J Hematol 106, 441–449 (2017). https://doi.org/10.1007/s12185-017-2252-y

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  • DOI: https://doi.org/10.1007/s12185-017-2252-y

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