Standard Treatment Options
Treatment Options Under Clinical Evaluation

Standard Treatment Options

Most investigators would recommend treatment according to the guidelines given above for standard treatment of children with Langerhans cell histiocytosis (LCH). Extensive or mutilating surgery to remove teeth or jaw bones is not indicated. Systemic chemotherapy will cause bone lesions to regress and the involved teeth and jaw bones can reform. Thalidomide and oral methotrexate have been effective in adults with skin disease.[1,2]

Results from LCH studies in children show that the rate of recurrent disease is appreciably reduced when adult patients receive 6 months of treatment with vinblastine and prednisone as opposed to single-agent treatment or attempts to irradiate multiple bone lesions.[3]

Anecdotal reports have described the successful use of the bisphosphonate pamidronate in controlling severe bone pain in patients with multiple osteolytic lesions.[4-6]

Another approach using anti-inflammatory agents (pioglitazone and rofecoxib) coupled with trofosfamide in a specific timed sequence was successful in two patients with disease resistant to standard chemotherapy treatment.[7]

There have been no published clinical trial results for adult LCH patients.

• The LCH-A1 trial for adult patients using vinblastine and prednisone with mercaptopurine is open for accrual. It should be noted that early toxicity data has shown that neuropathy secondary to vinblastine is frequent, so the protocol is in the process of being amended. For patients who do not respond to this front-line therapy, cladribine is effective for adults with skin, bone, lymph node, and probably pulmonary and central nervous system disease.[8-10]
  • Special considerations on the LCH-A1 trial for adult LCH patients with lung disease and who smoke cigarettes: Steroid efficacy for treating adult LCH is controversial because past case series reports on LCH patients with pulmonary disease did not control for smoking cessation. Most adult patients with LCH have gradual disease progression with continued smoking. The disease may regress or progress with the cessation of smoking.[11] In the LCH-A1 trial, patients are first offered a smoking cessation program and observation. If the smoking cessation program does not work, steroid treatment is started. Treatment of progressive disease after 6 months of steroids is up to the investigator’s choice, but usually includes vinblastine and mercaptopurine or 2-chlorodeoxyadenosine.

• Lung transplant may be necessary for adults with extensive pulmonary destruction from LCH.[12] A multicenter study documented a 54% survival at 10 years posttransplant with 20% of patients having recurrent LCH that did not impact survival; longer follow-up of these patients is needed.

For more information about LCH trials for adults, see the Histiocyte Society Website

References

1. McClain KL, Kozinetz CA: A phase II trial using thalidomide for Langerhans cell histiocytosis. Pediatr Blood Cancer 48 (1): 44-9, 2007.  [PUBMED Abstract]
   
   
2. Steen AE, Steen KH, Bauer R, et al.: Successful treatment of cutaneous Langerhans cell histiocytosis with low-dose methotrexate. Br J Dermatol 145 (1): 137-40, 2001.  [PUBMED Abstract]
   
   
3. Titgemeyer C, Grois N, Minkov M, et al.: Pattern and course of single-system disease in Langerhans cell histiocytosis data from the DAL-HX 83- and 90-study. Med Pediatr Oncol 37 (2): 108-14, 2001.  [PUBMED Abstract]
   
   
4. Arzoo K, Sadeghi S, Pullarkat V: Pamidronate for bone pain from osteolytic lesions in Langerhans'-cell histiocytosis. N Engl J Med 345 (3): 225, 2001.  [PUBMED Abstract]
   
   
5. Farran RP, Zaretski E, Egeler RM: Treatment of Langerhans cell histiocytosis with pamidronate. J Pediatr Hematol Oncol 23 (1): 54-6, 2001.  [PUBMED Abstract]
   
   
6. Brown RE: Bisphosphonates as antialveolar macrophage therapy in pulmonary langerhans cell histiocytosis? Med Pediatr Oncol 36 (6): 641-3, 2001.  [PUBMED Abstract]
   
   
7. Reichle A, Vogt T, Kunz-Schughart L, et al.: Anti-inflammatory and angiostatic therapy in chemorefractory multisystem Langerhans' cell histiocytosis of adults. Br J Haematol 128 (5): 730-2, 2005.  [PUBMED Abstract]
   
   
8. Saven A, Foon KA, Piro LD: 2-Chlorodeoxyadenosine-induced complete remissions in Langerhans-cell histiocytosis. Ann Intern Med 121 (6): 430-2, 1994.  [PUBMED Abstract]
   
   
9. Weitzman S, Wayne AS, Arceci R, et al.: Nucleoside analogues in the therapy of Langerhans cell histiocytosis: a survey of members of the histiocyte society and review of the literature. Med Pediatr Oncol 33 (5): 476-81, 1999.  [PUBMED Abstract]
   
   
10. Pardanani A, Phyliky RL, Li CY, et al.: 2-Chlorodeoxyadenosine therapy for disseminated Langerhans cell histiocytosis. Mayo Clin Proc 78 (3): 301-6, 2003.  [PUBMED Abstract]
    
    
11. Mogulkoc N, Veral A, Bishop PW, et al.: Pulmonary Langerhans' cell histiocytosis: radiologic resolution following smoking cessation. Chest 115 (5): 1452-5, 1999.  [PUBMED Abstract]
    
    
12. Dauriat G, Mal H, Thabut G, et al.: Lung transplantation for pulmonary langerhans' cell histiocytosis: a multicenter analysis. Transplantation 81 (5): 746-50, 2006.  [PUBMED Abstract]
    
    

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