Abstract
It is common knowledge that in β-thalassemic syndromes bone alterations are absent at birth, but may be appreciated radiologicallly after the 1st year of life and become characteristic during early childhood [3, 4,10,11, 41, 52, 55, 73, 82]. Bone marrow hyperplasia is generally considered responsible for skeletal changes [12, 14, 18, 27, 30, 50, 77] (osteopenia with widening of the medulla, thinning of the cortex in long bones and reactive periosteal new bone formation). These alterations, described by Cooley and Lee in 1925, are diagnostic and characteristic in the severe forms of anemia (homozygous β-thalassemia). They involve the entire skeleton but are usually more conspicuous in the skull and extremities [4, 10, 11, 41, 52, 53, 55]. With advancing age, skeletal lesions disappear in the peripheral segments, where red marrow is converted into yellow marrow, but they persist in the axial skeleton, where the bone marrow remains red. Therefore, in adolescence and early adulthood bone changes are particularly common in the skull, pelvis, and spine. Pseudotumors of extramedullary hematopoiesis may occur in paravertebral sites and in many other organs and tissues [8, 22, 45, 57, 58]. This process is far more frequent in thalassemia intermedia [13, 66].
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Scutellari, P.N., Franceschini, F., Orzincolo, C. (1989). A Reappraisal of Some Skeletal Changes in Currently Treated β-Thalassemia. In: Papavasiliou, C., Cambouris, T., Fessas, P. (eds) Radiology of Thalassemia. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-72587-6_6
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