Τίτλος:
Rare diseases of bone: Erdheim-Chester and Rosai-Dorfman non-Langerhans cell histiocytoses
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
▪ Non-Langerhans cell histiocytosis (N-LCH) summarizes a group of rare diseases with different clinical presentations, pathogenesis and morphology. These include primary cutaneous N-LCH, cutaneous N-LCH with systemic involvement, and primary extracutaneous systemic forms with occasional cutaneous involvement. ▪ The juvenile (JXG) and non-juvenile xanthogranuloma (N-JXG) family of histiocytoses are N-LCH: the JXG family consisting of the JXG (cutaneous), xanthoma disseminatum (cutaneous and systemic) and Erdheim-Chester disease (ECD; systemic); and the N-JXG family consisting of the solitary reticulohistiocytoma (cutaneous), multicentric reticulohistiocytosis (cutaneous and systemic) and Rosai- Dorfman disease (RDD; systemic). ▪ ECD is a clonal disorder from the JXG family of N-LCH; RDD is a reactive proliferative entity from the non-juvenile xanthogranuloma family of N-LCH. ▪ ECD and RDD N-LCH are rare disorders, which are difficult to diagnose, with multi-organ involvement including bone and systemic symptoms, and which respond to therapy in an unpredictable way. ▪ The key to successful therapy is accurate identification at tissue level and appropriate staging. Patients should be observed and monitored in a long-term pattern. Prognosis depends on disease extent and the organs involved; it is generally good for RDD disease and variable for ECD. © 2018 The author(s).
Συγγραφείς:
Mavrogenis, A.F.
Igoumenou, V.G.
Antoniadou, T.
Megaloikonomos, P.D.
Agrogiannis, G.
Foukas, P.
Papageorgiou, S.G.
Περιοδικό:
EFORT Open Reviews
Εκδότης:
British Editorial Society of Bone and Joint Surgery
Λέξεις-κλειδιά:
alpha interferon; anakinra; cladribine; corticosteroid; cyclophosphamide; cyclosporine; doxorubicin; infliximab; protein tyrosine kinase inhibitor; steroid; vemurafenib; vinblastine; vincristine, alopecia; arthralgia; Article; bone lesion; bone marrow suppression; bone scintiscanning; curettage; depression; disease classification; Erdheim Chester disease; fatigue; fever; gastrointestinal symptom; human; juvenile xanthogranuloma; myalgia; pathogenesis; pathology; positron emission tomography; priority journal; prognosis; reticulohistiocytosis; sinus histiocytosis; xanthoma
DOI:
10.1302/2058-5241.3.170047
