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. 2016 Jul 1;8(1):e2016033.
doi: 10.4084/MJHID.2016.033. eCollection 2016.

Bisphosphonates in Langerhans Cell Histiocytosis: An International Retrospective Case Series

Free PMC article

Bisphosphonates in Langerhans Cell Histiocytosis: An International Retrospective Case Series

Deepak Chellapandian et al. Mediterr J Hematol Infect Dis. .
Free PMC article


Background: Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH), which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH.

Method: We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation.

Results: Ten patients (77%) had a single system bone disease, and 3 (23%) had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years). Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve (92%) achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years). One patient did not respond. No major adverse effects were reported in this series.

Conclusion: Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted.


Figure 1
Figure 1
3-dimensional CT of mandible before (a) and after (b) 3 courses of pamidronate therapy. a) Osteolytic lesions in the ascending ramus, neck and condylar head of right mandible. b) Bone remodeling with thick periosteal reaction.
Figure 2
Figure 2
Skull CT before (a) and after (b) 2 courses of pamidronate therapy. a) Permeative bone lesions in the lateral wall of orbit and soft tissue mass involving the right lacrimal gland and lateral rectus muscle. b) Bone remodeling and reduction in orbital soft tissue mass

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