Multimodality imaging showing complete cardiovascular involvement by Erdheim-Chester disease.
Erdheim-Chester disease (ECD) is a multisystem non-Langerhans form of cell histiocytosis. Histiocytic infiltration leads to xanthogranulomatous infiltrates of multiple organ systems. Erdheim-Chester disease was first reported in 1930, only 320 cases reported in the literature. Cardiac involvement in ECD carries worst prognosis beside the central nervous system. We report the first case with pan-cardiac involvement diagnosed with multimodality imaging.
Alharthi
Mohsen S
MS
Cardiovascular Division, Internal Medicine Department, Mayo Clinic Arizona, Scottsdale, AZ, USA.
Calleja
Anna
A
Panse
Prasad
P
Appleton
Christopher
C
Jaroszewski
Dawn E
DE
Tazelaar
Henry D
HD
Mookadam
Farouk
F
D001011:Aorta / Q000473:pathology; D017338:Cladribine / Q000627:therapeutic use; D031249:Erdheim-Chester Disease / Q000150:complications* / Q000473:pathology; D005260:Female; D006325:Heart Atria / Q000473:pathology; D006331:Heart Diseases / Q000209:etiology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D010490:Pericardial Effusion / Q000175:diagnosis* / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D020519:Pericardiocentesis; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D017338:Cladribine