Langerhans cell histiocytosis
Consultation and second opinions for children with LCH and other histiocytic disorders, from the principal investigator of the international LCH-IV study.
About LCH
In Langerhans cell histiocytosis, certain cells of the immune system multiply and form lesions in different organs. It is most common in childhood but can occur at any age.
Bones, for example the skull, and the skin are most often involved. Less often LCH affects lymph nodes, liver, spleen, lungs, bone marrow or the pituitary gland. The course ranges from a single lesion to disease in several organ systems, and this determines the treatment.
What we offer
- Assessment of suspected LCH and planning of further diagnostics
- Second opinion on diagnosis, extent of disease and treatment plan
- Advice on international studies and current treatment options
- Coordination with St. Anna Children’s Hospital and St. Anna Children’s Cancer Research Institute
- Long-term follow-up and recognition of late effects
Possible signs
Symptoms depend on which organs are involved. They are nonspecific and usually have other, harmless causes. If they persist for weeks or keep coming back, a medical evaluation is advisable.
- Bonespain or a palpable swelling, especially of the skull, sometimes of the spine, pelvis or limbs
- Skina persistent scaly or crusted rash, for example on the scalp or in skin folds, that does not respond to usual treatment
- Earsrecurrent or persistent discharge from the ear
- Hormonesunusually strong thirst and large amounts of urine, a possible sign of diabetes insipidus
- Generalenlarged lymph nodes, liver or spleen, fever or failure to thrive, especially in young children
How LCH is diagnosed
LCH is confirmed on a tissue biopsy, usually from a bone or skin lesion. The pathologist identifies the typical cells with special stains. Testing for a specific gene mutation is often added.
Staging follows: blood and urine tests, ultrasound and, depending on the situation, X-rays, MRI or other imaging. This shows whether one or several organ systems are involved and whether organs such as the liver, spleen or bone marrow are affected. This classification determines the next steps.
Treatment and follow-up
Treatment depends on the extent and site of disease. It ranges from observation or a local procedure for single lesions to systemic therapy for extensive disease. The treating center decides together with the family which approach fits the individual situation.
It is based on international recommendations such as the Euro Histio Network guidelines for patients up to 18 years, co-authored by Prof. Minkov. Regular follow-up remains important after treatment, because the disease can recur and some consequences appear only later.
Questions about LCH
Is LCH a form of cancer?
LCH is now classified as a neoplasm, because the abnormal cells usually carry an acquired mutation in the MAPK signaling pathway. Its course varies widely: single bone lesions often heal with little treatment, while extensive disease needs treatment according to international standards.
How is LCH diagnosed?
The diagnosis is made on a tissue biopsy. Staging with blood tests and imaging then shows which organs are involved, and this determines the treatment.
Why is a second opinion helpful?
LCH is rare, and many doctors see only a few cases in their career. A second opinion can help to confirm the diagnosis, the extent of disease and the treatment plan.
Why is long-term follow-up important?
LCH can recur, and in some children consequences appear only years later, such as hormonal problems, changes in bones or teeth, or neurological symptoms. Regular follow-up helps to detect such late effects early.
Can families from abroad request a consultation?
Yes. Consultations are available in English, German, Bulgarian and Russian. Before the appointment we clarify which records are needed and whether a visit in person is necessary.
Questions about LCH?
Call us or send a short written request. We will tell you which records we need.