Scutellari P N, Bighi S, Franceschini F, Orzincolo C
Minerva Med. 1982 Mar 3;73(9):457-66.
Bone changes of varying degree and distribution were observed by the Authors in 10 carriers of beta-thalassaemia intermedia in skull, ribs, hand, elbow and knee bones. When they are present, skeletal changes are the same of homozygous beta-thalassaemia, but less marked and spread. Instead, symptomatology is more homogeneous, usually characterized by mild anaemia and morphological changes of red cells with haemoglobin values in the 6-11 g/dl range, splenomegaly, jaundice, gallstones, crural ulcers. The clinical state goes through the asymptomatic condition to a more severe condition with typical appearance of Cooley's anaemia.
作者在10例中间型β地中海贫血患者的颅骨、肋骨、手部、肘部和膝部骨骼中观察到不同程度和分布的骨骼变化。当出现骨骼变化时,其与纯合子β地中海贫血相同,但程度较轻且范围较局限。相反,症状表现更为一致,通常特征为轻度贫血、红细胞形态改变、血红蛋白值在6-11g/dl范围内、脾肿大、黄疸、胆结石、小腿溃疡。临床状态从无症状状态发展到更严重的状态,出现典型的库利贫血外观。