Childhood cancer: Langerhans histiocytosis (histiocytosis X)

Immune cells, histiocytes, multiply uncontrollably.
In Langerhans cell histiocytosis (also called "Histiocytosis X" referred to) there is an excessive, malignant proliferation of a particular cell type of the immune system, the so-called histiocytes. This increase can occur in various organs and tissues. The statistical frequency of Langerhans cell histiocytosis is 0.4 cases per 100,000 people.


There are three forms of Histiocytosis X.
Langerhans cell histiocytosis can be divided into 3 subtypes:

* Eosinophilic granuloma: a manifestation of the disease at one or more locations in the bones.
* Hand-Schuller-Christian disease: occurrence of the disease (especially in infants) among others to the eyes, bones, gums, in the external ear canal and on the genitals.
* Disease Abt-Letterer-Siewe: It is (usually in infants and young children) to fever, skin lesions, foci in the bones and the lymphatic vessels as well as enlargement of liver and spleen.


Diagnostic procedures:
First indications of the presence of Langerhans histiocytosis arising from the complaints and the findings on physical examination. Further evidence to provide blood and urine tests, x-rays and magnetic resonance imaging and scintigraphy, continue to histological examination of tissue, such as bones from affected areas or from the skin.


Treatment depends on the nature of the disease.
The treatment is a function of each subtype present. Thus it is possible for a limited eosinophilic granuloma of the bone region, remove the "Knochenherd" operatively. If this district, however, are in a region that is less well suited for an operation) (eg, located very close to a joint that is a candidate radiotherapy. In a eosinophilic granuloma, which manifests itself in several places of the skeleton, the implementation of chemotherapy is more meaningful. This also applies to the disease Hand-Schuller-Christian disease and Abt-Letterer-Siewe that occur tend to be scattered in the body and are less limited regionally.


The forecast is - especially in individual herds bones - good.
The prognosis of Langerhans cell histiocytosis is especially good if only a single "Knochenherd" is present. But is also in the other forms, the cure rate averages 70 percent. However, the cure rate in young children (under 3 years), 50 percent is slightly lower.