Histiocytosis

Histiocytosis refers to a group of rare tumour pathologies characterised by the accumulation of histiocytes (a subgroup of immune system cells) in various tissues. This group of diseases includes, most notably,  Langerhans cell histiocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and malignant histiocytoses and cutaneous histiocytoses.  

Frequently affected organs are the skin, bones, lungs, pituitary gland, the central nervous system, the heart, the large blood vessels and the retroperitonium.

The diagnosis is based on microscopic and genetic analyses carried out on biopsies of the affected organs.  Due to the rarity of these diseases and the sometimes difficult anatomopathological analysis, consultation is required between the reference centres for the purposes of diagnosis and treatment.   

A multidisciplinary approach is adopted for the treatment of histiocytosis. The treatment will depend on the type of histiocytosis and the seriousness of the affected organs  

Close monitoring without treatment can sometimes be proposed. In some situations, the use of analgesic medicines and bone consolidation succeeds in managing symptoms.  

For certain localised bone or cutaneous forms of histiocytosis, surgery and radiotherapy enable the disease to be managed or even cured. In other more serious situations it can be necessary to have recourse to the use of corticoids or chemotherapy.  

In 2010, the discovery of genetic anomalies in cases of histiocytosis made it possible to improve significantly the treatment of severe forms thanks to the use of targeted therapies.  

The often chronic nature of these diseases justifies regular monitoring by a specialist in the field of histiocytoses.    
 

Useful links

Association Histiocytose France et Groupe d’Etude des Histiocytoses : Histiocytose.org - Histiocytose Langheransienne - histiocytose.org

Filière de santé Maladies Rares Immuno-Hématologiques (MARIH) : Accueil - MaRIH - Filière de santé Maladies Rares Immuno-Hématologiques

Patients with histiocytosis sometimes have a long waiting period before obtaining a firm diagnosis. Please feel free to contact our team regarding a diagnosis and benefit from a medical assessment.

Our department is actively involved in  number of protocols for translational and clinical research (expression of BCL2 in histiocytoses, in cooperation with the French reference centre for histiocytoses, under the supervision of Professor Julien Haroche.

Do not hesitate to talk to your haematologist.