Understanding Langerhans cell histiocytosis to avoid the many complications, support healthy development

Langerhans cell histiocytosis (LCH) is a rare disorder that is of special interest to the Pediatric Head and Neck Cancer Foundation, as it tends to affect young boys and often arises in the head and neck region.

What is this disorder exactly?

Let’s break it down. Histiocytosis refers to a group of rare blood disorders. With these conditions, your body makes histiocytes, which are abnormal white blood cells. These histiocytes cause inflammation and can damage your organs. While there are more than 100 types of histiocytosis, LCH is one of the most common forms. It affects the Langerhans cells. These white blood cells support healthy immune function. They help to fight off infections. However, when these cells build up, they can cause tissue damage and lead to the formation of lesions and tumors throughout the body.

Who is at greatest risk of LCH?

LCH most commonly affects babies and children. On average, pediatric patients were diagnosed at six-and-a-half years of age. It also has a predilection for young males. Your child may also be at risk of developing LCH if there is a history of it in your family or if they had infections as a newborn. A lack of vaccinations in childhood is also a risk factor, as is exposure to certain chemicals and environmental toxicants during pregnancy. In about half of cases, a random genetic mutation in the developing fetus is to blame. This BRAF gene controls cell development. With this mutation, the gene causes too many LCH cells to grow and divide.

What parts of the body does LCH affect?

As white blood cells, LCs can be found throughout the body. However, the head and neck are a common subsite. Eighty percent of cases have skeletal involvement, with the skull representing the bone most likely to be affected by LCH.

What are the most common symptoms?

Since so many organs and bodily systems can be involved, LCH symptoms vary significantly depending on the affected areas. A few of the HN-related symptoms include:

  • Swelling or lumps over eye sockets, jawbones, ear bones, and on the skull
  • Headaches
  • Neck pain
  • Fractures
  • A scalp rash that resembles cradle cap
  • Mouth sores
  • Wobbly teeth
  • Inflamed gums
  • Chronic ear infections
  • Ear pain
  • Discharge from the ear canal
  • Bulging eyes
  • Vision impairment

Prompt and appropriate treatment of severe LCH critical. It’s estimated that almost half of children will experience severe complications due to untreated LCH. These complications range from delays in growth and musculoskeletal disabilities to hearing loss and the development of leukemia, lymphoma, and other cancers.

What is considered to be the standard treatment?

The answer to this question depends on where Langerhans cells have built up and if these sites are considered “low-risk” or “high-risk.” We must also account for whether your child’s LCH is characterized as a “single system” (when one organ or part of the body is involved) or a “multi-system” (when at least two organs or systems of the body are affected). Depending on your child’s needs, corticosteroids may be recommended to alter the function of the white blood cells, which, in turn, affects the LCH cells. We may surgically resect or remove tumors and surrounding tissues. Chemotherapy, radiation, immunotherapy, and targeted therapy to block the proteins responsible for LCH growth are also options that may benefit your child.

Based on your child’s specific risk and affected site(s), you may be advised to exercise “watchful waiting” to ensure LCH does not spread or recur. LCH can improve without intervention, usually in single-system cases affecting the skin and bone.

Do not hesitate to contact the Pediatric Head and Neck Cancer Foundation if you have concerns about changes regarding your child. If you want to get involved in our fight to combat this crisis of childhood HN cancers, contact us today at pediatricheadneckcancer@gmail.com We welcome you to our community!