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How We Treat Histiocytosis

  • The treatment of adult histiocyte disorders is highly personalized and highly individualized – and requires clinicians who have deep expertise in the disease, such as the specialists in the Adult Histiocyte Disorders Center. Unlike most cancers, histiocyte disorders do not have a stage. Instead, we determine which sites are affected, including whether:

    • Only one site is affected (solitary-site disease),
    • Multiple sites are affected but in the same organ (solitary-system disease), or
    • Multiple organs are affected (multisystem disease).

    For referring physicians...

    Because the patient's primary care physician or community specialist is an integral part of the patient's care team, we are committed to collaborating with you in the care of your patient.

    If you are a referring physician and have a patient with a question of histiocyte disorder, we look forward to working with you.

    Find out more about how to refer a patient to DF/BWCC.

    Find out about Physician Gateway, our portal that helps you keep track of patients you have referred for care. Physician Gateway is available for primary care providers, referring providers, and your authorized office staff.

    The various sites affected by histiocyte disorders may carry different risks and require different treatments.

    Treating Langerhans cell histiocytosis (LCH)

    Lung involvement

    LCH that occurs only in the lungs of smokers can sometimes be treated by quitting smoking. Often with this simple intervention, which has many other positive health benefits, the disease will go away.

    If patients are not able to quit smoking, or if the disease persists after the patient quits smoking, a course of prednisone may be required in addition to inhaler medications that are used for asthma or emphysema. In this case we enlist the help of our lung specialists.

    Occasionally, these steps do not work and chemotherapy is needed. Rarely, lung LCH can become severe enough to require a lung transplant, although this is typically in patients who continue to smoke.

    Solitary-site LCH

    Solitary-site LCH can often be treated with surgical removal alone, or with steroid injections into the affected area. Occasionally, radiation is needed, but this is less common. The Adult Histiocyte Disorders Center's experts in radiation oncology are familiar with the treatment of histiocyte disorders. Chemotherapy is rarely needed for solitary-site LCH.

    Solitary-system and multisystem disease

    Solitary-system and multisystem disease often require chemotherapy. If chemotherapy treatments are needed, they are almost always done on an outpatient basis. The choice of appropriate chemotherapy depends on many factors, including the location of the disease and your overall health. Therefore, a high level of expertise in managing LCH and administering chemotherapy is critical.

    Expanded treatment options

    The recent identification of BRAF mutations in LCH has expanded our treatment options:

    • There are now oral medications approved by the FDA to treat melanoma, a cancer that also has this mutation. These pills can be effective, but can have side effects on the liver and skin. Thus, for patients who are using these treatments, we always work with a Center nephrologist and dermatologist, who have expertise in the side effects of these agents.
    • Drugs that inhibit molecules such as MEK are also being investigated.

    Clinical trial

    Dana-Farber is conducting a research study of the chemotherapy drug Lenalidomide as a possible treatment for LCH, ECD, or HS.

    Treating Erdheim-Chester disease (ECD)

    Some patients have their ECD diagnosed incidentally while having radiology tests for other reasons, and in some cases, the disease may not require immediate treatment.

    ECD has historically been treated with interferon given once or three times per week indefinitely. Because interferon can have side effects, patients are monitored closely by our oncology team and other specialists.

    As with LCH, in cases of ECD where a BRAF mutation is present, pills that inhibit this mutation may be used, with the same vigilance regarding possible skin and other side effects from these agents. Chemotherapies are also sometimes used. Weighing the risks and benefits of each option requires significant expertise.

    Treating histiocytic sarcoma (HS)

    The treatment of HS is less well defined, since it is such a rare disease. Surgical removal and radiation are preferred, if possible. If surgery and/or radiation are not possible, or if the disease comes back after these treatments, chemotherapy is generally the next step.

    Adult Histiocytosis Support Services and Follow-Up Care

    At the Adult Histiocyte Disorders Center, we understand that a histiocytosis diagnosis can be emotionally challenging, and that treatment can be physically demanding. When you become a patient at Dana-Farber/Brigham and Women's Cancer Center (DF/BWCC), our medical, nursing, and support teams make sure you receive the best and most complete care every step of the way.

    As a patient, you, your loved ones, and your family have access to an extensive array of support programs and services that address issues you may face before, during, and after your treatment. And to stay connected, join Dana-Farber's online Adult Histiocytosis Community on CancerConnect. There, you can safely express your thoughts, pose questions, voice concerns, and read what others have to say.

    Support Services at Dana-Farber/Brigham and Women's Cancer Center

    For a full list of our support services, as well as contact information, download our Supportive Resources brochure.