Cargando…

Primary myelofibrosis: spectrum of imaging features and disease-related complications

Primary myelofibrosis is a chronic clonal stem cell disorder that results in a build-up of marrow fibrosis and dysfunction, hypermetabolic states, and myeloid metaplasia. The clinical and radiological consequences can be quite diverse and range from the manifestations of osteosclerosis and extramedu...

Descripción completa

Detalles Bibliográficos
Autores principales: Oon, Sheng Fei, Singh, Dalveer, Tan, Teng Han, Lee, Allan, Noe, Geertje, Burbury, Kate, Paiva, Joseph
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6684717/
https://www.ncbi.nlm.nih.gov/pubmed/31388788
http://dx.doi.org/10.1186/s13244-019-0758-y
Descripción
Sumario:Primary myelofibrosis is a chronic clonal stem cell disorder that results in a build-up of marrow fibrosis and dysfunction, hypermetabolic states, and myeloid metaplasia. The clinical and radiological consequences can be quite diverse and range from the manifestations of osteosclerosis and extramedullary haematopoiesis to thrombohaemorrhagic complications from haemostatic dysfunction. In addition, there is the challenge of identifying less well-recognised sites of extramedullary haematopoiesis and their site-specific complications. The intent of this article is to illustrate the spectrum of primary myelofibrosis as declared though multimodality imaging, with examples of both common and rarer disease manifestations.