Histiocytosis

At Children’s Mercy, we specialize in diagnosing and treating rare childhood diseases — including histiocytosis. Whether your child has just been diagnosed or you’re searching for answers, our team of pediatric experts will work closely with your family to create a personalized care plan that meets your child’s unique needs.

We’re proud to be one of the few centers in the nation with the expertise and resources to care for children with all forms of histiocytosis, and we’re here to guide your family every step of the way.

What is Histiocytosis 

Histiocytosis is a rare group of diseases that happen when the body makes too many histiocytes—a type of white blood cell that normally helps fight infection. When these cells build up in the body, they can cause inflammation and damage to organs and tissues.

There are several types of histiocytosis, and each one can affect children differently:

• Langerhans Cell Histiocytosis (LCH):This is the most common type. It can cause skin rashes, bone pain, or even affect the lungs and other organs.
• Hemophagocytic Lymphohistiocytosis (HLH):A more aggressive form that can cause fever, enlarged organs, and serious inflammation throughout the body.
• Sinus Histiocytosis with Massive Lymphadenopathy:A rare condition that causes swollen lymph nodes, often in the neck.
• Juvenile Xanthogranuloma:A benign lump or bump caused by a buildup of immune cells, that usually goes away on its own over time.
  
  

What Causes It?

In many cases, the exact cause is unknown. Some forms may be linked to genetic changes or problems with the immune system.

How is it Treated?

Treatment depends on the type and severity of the disease. It may include:

• Steroids or chemotherapy to reduce inflammation
• Targeted therapies for specific genetic mutations
• Supportive care to manage symptoms

What to Expect 

We understand that a diagnosis of histiocytosis can feel overwhelming. Our goal is to support your family every step of the way — from the first appointment to long-term follow-up care.

Your First Visit

During your first visit, you’ll meet with a team of pediatric specialists, including hematologists/ oncologists, nurse practitioners and social workers. We’ll review your child’s medical history, perform a physical exam, and may order tests such as:

• Blood work
• Imaging (like X-rays or MRI)
• Biopsy (if needed)

We’ll explain everything clearly and answer all your questions.

Personalized Treatment Plan

Once we have a diagnosis, we’ll create a treatment plan tailored to your child’s specific type of histiocytosis. This may include:

• Medications (such as steroids or chemotherapy)
• Targeted therapies, including collaboration with pediatric specialists like orthopedic surgeons, endocrinologists, dermatologists or pathologists.
• Supportive care (pain management, nutrition, etc.)
  
  

Ongoing Care and Monitoring

Treatment may take place over weeks or months, depending on the type and severity of the disease. We’ll schedule regular follow-ups to monitor progress and adjust the plan as needed.

You’ll also have access to:

• Child life specialists to support emotional wellbeing
• Social workers and financial counselors
• Educational resources to help your child stay on track