Type:
Educational Exhibit
Keywords:
Bones, Lung, Paediatric, CT, Digital radiography, MR, Chemotherapy, Surgery, Pathology
Authors:
G. Sapouridis1, M. Sidiropoulou1, G. Tsamourliadou1, A. MARKOU2, A. Baltatzidis1, E. Papakonstantinou3, D. Koliouskas1, A. Anastasiou1; 1Thessaloniki/GR, 2FLORINA/GR, 3Thessaloniki /GR
DOI:
10.1594/ecr2018/C-1828
Background
Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation and migration of dendritic antigen-presenting histiocytes,
a form of white blood cell found in healthy people that is supposed to protect the body from infection.
In people with LCH,
these cells multiply excessively and are capable of infiltrating almost any tissue or organ,
causing tumors called granulomas to form.
The etiology of LCH is unknown.
It may manifest at any age usually children between 1 and 3 years.
Aggressive forms of the disease are mainly found in younger children.
LCH is divided into three groups on the basis of the number of lesions and systems involved
- The unifocal form (70% of LCH cases) is limited to a single bone or a few bones and may involve the lung.
Patients usually present between 5 and 15 years of age.
- The multifocal unisystem form (20% of LCH cases) involves multiple bones as well as the reticuloendothelial system (liver,
spleen,
lymph nodes,
and skin).
It is often accompanied by diabetes insipidus when the pituitary gland is involved.
Patients with this form of disease present earlier than those with unifocal disease,
typically between 1 and 5 years of age.
- The multifocal multisystem form (10% of LCH cases) often is fatal.
It typically is diagnosed in the first 2 years of life and is characterized by disseminated involvement of the reticuloendothelial system,
anemia,
and thrombocytopenia.
Eosinophilic granuloma,
Hand-Schüller-Christian disease,
histiocytosis X,
and Letterer-Siwe disease are older names for the three groups that were used when LCH was thought to be several different diseases; these names should now be disregarded.
Patients may be asymptomatic or may present with focal pain and swelling and are at risk for pathologic fractures.
Diagnosis of LCH is made by clinical features,
histopathology,
immunohistochemistry,
imaging findings and is corroborated by biopsy.
The radiologic features of LCH are categorized by individual organ systems Fig. 1