Arterial elastorrhexis in beta-thalassaemia intermedia, sickle cell thalassaemia and hereditary spherocytosis

Arterial and stromal elastorrhexis, an elastic tissue disorder, was recentl y described in beta -thalassaemia major. Histopathological material from 10 patients with thalassaemia intermedia, 14 with sickle cell thalassaemia an d 18 with hereditary spherocytosis was examined in order to investigate the specificity of the arteriopathy. Histological re-examination was made in a total of 42 spleens with parasplenic lymph nodes in 14 cases. 26 surgical liver biopsies and 16 gallbladders with associated regional lymph nodes. Ar teriopathy, qualitatively similar to that seen in beta -thalassaemia major, was found in up to 90% of extrasplenic muscular arteries. Elastorrhexis le sions were also found in intrasplenic arteries and in stromal elastic tissu e of spleens and parasplenic lymph nodes, in the absence of tissue iron ove rload. The arteriopathy appears in the first decade of life even in spleens of normal weight, and seems unrelated to the severity of permanent anaemia . It is suggested that patients suffering from hereditary chronic haemolyti c diseases are subject to an elastic tissue disorder which is similar to he reditary pseudoxanthoma elasticum, the earliest and most frequent manifesta tion of which is arterial elastorrhexis of muscular extrasplenic arteries.