Langerhans Cell Histiocytosis

Also known as: LCH, childhood Langerhans cell histiocytosis, diffuse reticuloendotheliosis, eosinophilic granuloma , histiocytosis x and others.

What is Langerhans cell histiocytosis?

Langerhans cell histiocytosis (LCH) is a rare disease of children (most commonly between the ages of 1-3 years; boys more often than girls) in which a certain immature normal white blood type (Langerhans cells, a type of histiocyte) grows uncontrollably and rapidly in almost any organ; there appears to be debate as to whether this is a form of cancer or not.

What causes Langerhans cell histiocytosis? 

The cause is unknown, however gene mutations have been found in about half the patients.

What are the symptoms of Langerhans cell histiocytosis? 

Symptoms depend on which tissue(s) or organ(s) the cell multiplies in. They include:

  • fever
  • weakness and tiredness
  • bone involvement (78% of patients) which often presents as swelling or a lump, or fractures of bone that occur for no reason
  • skin rashes (50% of patients)
  • lung damage (20-40% of patients)
  • swollen lymph nodes (30% of patients)
  • ear infections
  • liver problems
  • and many others.

What are Langerhans cell histiocytosis care options?

Children with localized disease may require no treatment. In some cases, surgery to remove the tumor, low-dose radiation therapy and chemotherapy may be utilized.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: April 04, 2022 04:25 PM

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