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Langerhans Cell Histiocytosis Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Adult LCH


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.[9]

Treatment of single-system skin disease

  • Localized lesions can be treated by surgical excision, but as with bone, mutilating surgery, including hemivulvectomy, should be avoided unless the disease is refractory to available therapy.
  • Topical therapies are described in greater detail in the childhood isolated skin involvement section of this summary and include topical or intralesional corticosteroid, topical tacrolimus, imiquimod, and psoralen and long-wave ultraviolet radiation (PUVA). Therapies such as PUVA may be more useful in adults where long-term toxicity may be less of a consideration.[10,11,12]
  • Systemic therapy for severe skin LCH includes oral methotrexate, oral thalidomide, oral interferon-alpha, or combinations of interferon and thalidomide.[13,14] Recurrences after stopping treatment may occur but may respond to retreatment.
  • Oral isotretinoin has achieved remission in some refractory cases of skin LCH in adults.[15]

Chemotherapy for the treatment of single-system and multisystem disease

Chemotherapy is generally used for skin LCH associated with multisystem disease in adults.

  • A single-center, retrospective review of 58 adult LCH patients reported on the efficacy and toxicities of treatment with vinblastine/prednisone, cladribine, and cytarabine. Patients treated with vinblastine/prednisone had the worst outcome, with 84% not responding within 6 weeks or relapsing within a year. The no-response/relapse rate was 59% for cladribine and 21% for cytarabine. Grade 3 or 4 neurotoxicity occurred in 75% of patients treated with vinblastine. Grade 3 or 4 neutropenia occurred in 37% of patients treated with cladribine and in 20% of patients receiving cytarabine.[16]
  • Etoposide has been used with some success in single-system and multisystem LCH. Use of prolonged oral etoposide in adults with skin LCH has been reported with minimal toxicity, while 3-day courses of intravenous etoposide 100 mg/m2 /day achieved complete remission in a small number of patients with resistant single-system and multisystem disease.[17] Another study at the same center found that azathioprine was the most successful drug for localized disease in adults with the addition of etoposide for refractory and multisystem disease.[18]
  • For patients who do not respond to front-line therapy with etoposide, 2-CdA is effective for adults with skin, bone, lymph node, and probably pulmonary and central nervous system (CNS) disease.[19,20] The first study that used 2-CdA to treat refractory and recurrent skin LCH disease reported on three patients (ages 33, 51, and 57 years) who received two to four courses of 2-CdA at 0.7 mg/kg intravenously over 2 hours/day for 5 days.[19] In a series of five adults (one untreated and four with refractory LCH treated with 2-CdA at the same dose noted above), three patients achieved a complete remission and two patients achieved a partial remission.[20]
  • An adult lymphoma treatment regimen, MACOP-B (methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone and bleomycin), was used in three patients with multisystem LCH and four with single-system multifocal bone LCH from 1995 to 2007.[21] Total duration of therapy was 12 weeks, response was seen in all patients, two with partial response and five with complete response. Three recurrences were seen after stopping therapy.[21] Despite the small number of patients and the retrospective nature of the study, MACOP-B may be useful as salvage therapy in adult patients with LCH and deserves further study.[22]
  • Anecdotal reports have described the successful use of the bisphosphonate pamidronate in controlling severe bone pain in patients with multiple osteolytic lesions.[4,5,6]
  • Imatinib mesylate has been effective in the treatment of four adult LCH patients who had skin, lung, bone, and/or CNS involvement.[23,24] Another adult LCH patient did not respond to imatinib mesylate.[25]
  • A case report suggests some benefit to treating neurodegenerative CNS LCH disease with infliximab, a tumor necrosis factor-alpha inhibitor.[26]
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