Langerhans Cell Histiocytosis

Langerhans cell histiocytosis causes an overgrowth of immune system cells called histiocytes —specifically Langerhans cells. These immune cells help your body fight off infections and destroy foreign invaders like fungi, viruses and bacteria.

When your body makes too many Langerhans cells, they can form lesions, most often on your bones. But they can also affect your skin, lungs, liver, spleen, teeth and gums, ears, eyes and nervous system.

Langerhans cell histiocytosis is considered an inflammatory neoplasm — technically neither an autoimmune condition nor cancer. Instead, it’s considered an abnormal growth of tissue that can be either benign (noncancerous) or malignant (cancerous). Often, LCH is passed down through families in their genes (inherited).

While most cases of LCH affect kids younger than 15, adults can also develop it. Not many providers are familiar with this condition in adults — but our expert team is.

What to expect at your first visit

When you come to your first appointment, you’ll do something important for us — share your story. We want to hear all about what brought you here. You can expect your provider to ask questions like:

• How have you been feeling?
• What kind of symptoms have you noticed?
• How long have you had these symptoms?
• How are these symptoms affecting your life?
• What other health conditions do you have?
• Has anyone in your family had health problems?

Because LCH can affect many body systems, it’s important to tell us about anything that hasn’t felt right. Hearing what’s been happening from your point of view can help our team build a treatment plan around you — not just your test results and diagnosis.

While you’re here, you’ll also have a physical exam so we can look for signs of Langerhans cell histiocytosis. Your provider will also order tests to help confirm a diagnosis. You may have:

Blood tests

Langerhans cell histiocytosis can cause low blood cell levels. So, we’ll do a complete blood count (CBC) to see how many red blood cells, white blood cells and platelets your body produces. Blood tests can also tell us how well your kidneys, liver and thyroid are working.

Biopsies

If you have an LCH tumor, we’ll take a sample of tissue from the tumor for a biopsy to see if it has a large amount of Langerhans cells or other signs of this condition. We may also do a bone marrow biopsy to see what’s going on in your bone marrow, bones and blood.

Imaging tests

Imaging exams let us look closely at your bones and soft tissues. We can use these to spot LCH tumors and cracks or breaks in your bones (fractures) caused by these tumors. You may have:

• X-rays.
• CT scans.
• MRIs.
• Positron emission tomography (PET) scans.
• Ultrasounds.

Second Opinions for Langerhans Cell Histiocytosis

Hearing you have a rare blood condition can leave you with a lot of questions. And make you want to be sure you’re getting the best possible care from providers that you trust. That’s why we encourage second opinions.

When you contact us, we’ll help you get an appointment as soon as possible. You’ll meet our expert hematology and oncology team to learn more about your diagnosis — and what to expect from treatment. Getting a second opinion helps you stay in charge of your health so you can feel confident about taking the next step.

Meet Our Langerhans Cell Histiocytosis Team

If you choose to come to Cleveland Clinic for your diagnosis and treatment, you’ll benefit from our team-based approach to care. We handpick a care team of providers based on your symptoms and how familiar they are with treating LCH, like:

• Hematologists.
• Oncologists.
• Dentists.
• Dermatologists.
• Gastroenterologists.
• Ophthalmologists.
• Orthopaedists.
• Otolaryngologists.
• Pathologists.
• Pulmonologists.
• Radiologists.
• Nurse practitioners.
• Physician assistants.
• Care coordinators.

All providers on your team will work together to confirm your diagnosis and build the best, most personalized treatment plan.

How we treat you for Langerhans cell histiocytosis depends on your symptoms and where it affects your body. Mild forms in only one body area (localized) may not need treatment — at least not right away. Instead, we may do “watchful waiting.” This means we keep a close eye on your condition and do regular tests to see if things are progressing.

Severe or widespread (disseminated) LCH affecting many areas requires treatment to lessen symptoms and prevent damage to your bones and organs. You may have:

Medications

We may prescribe drugs like corticosteroids and immunosuppressants to reduce inflammation and skin irritation. This is the most common treatment.

Systemic therapy

Depending on how severe the condition is, we may also use treatments that kill or keep abnormal Langerhans cells from growing and multiplying, like:

• Chemotherapy.
• Targeted therapy.
• Photodynamic therapy.

Rarely, you may have radiation therapy to shrink LCH tumors, but it’s not usually frontline treatment. The same goes for a stem cell transplant to replace abnormal blood cells with healthy stem cells from a donor. Your care team will share options with you and explain why they’re making these recommendations.

Surgery

Sometimes, your care team may decide to do surgery to remove LCH tumors, particularly if they’re affecting your bones. Your providers will go over what you can expect from surgery and recovery.

Life After Langerhans Cell Histiocytosis Treatment

Once you complete LCH treatment, you’ll move into follow-up mode. This means you’ll regularly have tests and see your care team to make sure your condition doesn’t come back (recur). And if it does, regular follow-up care means we can catch it and start treatment right away.

Taking the Next Step

Finding out you have a rare blood condition like Langerhans cell histiocytosis can instantly change your life. It can be a lot to process. But you can feel more secure knowing there’s an expert team of compassionate healthcare providers by your side. At Cleveland Clinic, we know what it takes to treat this rare condition — and we’ll stay with you well after treatment ends.