Histiocytosis Treatment & Management

Updated: Feb 14, 2017
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Treatment

Medical Care

Optimal treatment of Langerhans cell histiocytosis (LCH) has not been established. In ideal cases, the differences between normal cells and pathologic Langerhans cells (PLCs) should be used to guide treatment of the disease. However, a lack of sufficient information has hampered specific therapy.

Substantial variation of the disease and the fact that 10-20% of patients achieve spontaneous regression complicate comparisons of current nonspecific therapies. [259, 260, 261] Several agents, including drugs for cancer chemotherapy, have been effective in the treatment.

A study of by Rigaud et al of 1478 patients with Langerhans cell histiocytosis found that following a change in therapeutic strategy—an increase in the treatment period from 6 to 12 months, the use of repeated induction therapy in patients who responded poorly to initial induction with vinblastine and steroids, and the treatment of refractory disease in a risk organ with cladribine and cytarabine—the 5-year survival rate improved from 92% to 99%. In the specific group of patients with refractory disease in a risk organ, the 5-year survival rate increased from 60% to 92%. [262]

A study by Duan et al indicated that in adult patients with either multisystem or multifocal single-system Langerhans cell histiocytosis, treatment with either vindesine and prednisone or cyclophosphamide, etoposide, vindesine, and prednisone was similarly effective. The study, which involved 45 patients, also found high disease recurrence and the need for second-line therapy associated with the two regimens. [263]

Some suggest that treatment of Langerhans cell histiocytosis should be conservative and limited to individuals with constitutional symptoms, such as pain, fever, failure to thrive, and vital organ disorder as well as those with CNS risk involvement. [264] Treatment of these disorders must often be tailored to the patient’s prognostic factors, such as the patient’s age, extent of the disease, sites of involvement, and complications. For example, the biopsy and curettage are adequate treatment for a solitary bone lesion may be adequate therapy. [41, 265, 266, 267, 268]

See the Medication section for a discussion of agents used in the treatment of Langerhans cell histiocytosis.

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Consultations

Multidisciplinary care is essential for all patients. Consultation with an oral surgeon and an otolaryngologist, among others, may be required.

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