[Radiological appearance of bone manifestations in acute drepanocytic syndromes in adults (author's transl)].
The authors describe 18 cases of this syndrome and emphasize that attenuated clinical forms of homozygotic drepanocytosis exist, as well as, in a more general manner, in acute drepanocytic syndromes. The disease may first appear in adult life. The frequency of radiological signs in the spine has to be stressed, and the authors emphasize the diagnostic value of the "staircase" deformation of the vertebrae, representing the sequelae of bone ischemic lesions. The discovery of this radiological sign suggested the diagnosis of a hemoglobinopathy in two cases. This attenuated form of the disease presents the same risks of thromboses but these complications can be reduced by prophylactic treatment. This underlines the need for early detection and radiological investigation can be of great assistance.
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D001847:Bone Diseases / Q000000981:diagnostic imaging* / Q000209:etiology; D001853:Bone Marrow / Q000473:pathology; D006801:Humans; D006965:Hyperplasia / Q000000981:diagnostic imaging; D010019:Osteomyelitis / Q000000981:diagnostic imaging; D010020:Osteonecrosis / Q000000981:diagnostic imaging; D011859:Radiography; D012805:Sickle Cell Trait / Q000150:complications*; D013131:Spine / Q000000981:diagnostic imaging
Manifestations radiologiques osseuses des syndromes drépanocytaires majeurs chez l'adulte.