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International Journal of Hematology

, Volume 106, Issue 3, pp 441–449 | Cite as

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

  • Seong Wook Lee
  • Hyery Kim
  • Jin Kyung Suh
  • Kyung-Nam Koh
  • Ho Joon Im
  • Hee Mang Yoon
  • Jong Jin SeoEmail author
Original Article

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.

Keywords

Langerhans cell histiocytosis Spine Magnetic resonance imaging 

Abbreviations

LCH

Langerhans cell histiocytosis

MRI

Magnetic resonance imaging

Notes

Compliance with ethical standards

Funding

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

Conflict of interest

The authors declare that they have no competing interest.

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Copyright information

© The Japanese Society of Hematology 2017

Authors and Affiliations

  • Seong Wook Lee
    • 1
  • Hyery Kim
    • 2
  • Jin Kyung Suh
    • 3
  • Kyung-Nam Koh
    • 2
  • Ho Joon Im
    • 2
  • Hee Mang Yoon
    • 4
  • Jong Jin Seo
    • 2
    Email author
  1. 1.Department of PediatricsKorea University Ansan Hospital, Korea University College of MedicineSeoulKorea
  2. 2.Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children’s HospitalUniversity of Ulsan College of MedicineSeoulRepublic of Korea
  3. 3.Department of PediatricsKyungpook National University HospitalDaeguKorea
  4. 4.Department of Radiology and Research Institute of Radiology, Asan Medical CenterUniversity of Ulsan College of MedicineSeoulKorea

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